National Health (Highly Specialised Drugs Program) Special Arrangement Amendment (December Update) Instrument 2022 (Cth)

Case

PB 114 of 2022

National Health (Highly Specialised Drugs Program) Special Arrangement Amendment (December Update) Instrument 2022

National Health Act 1953

I, NIKOLAI TSYGANOV, Assistant Secretary (Acting), Pricing and PBS Policy Branch, Technology Assessment and Access Division, Department of Health and Aged Care, delegate of the Minister for Health and Aged Care, make this Instrument under subsection 100(2) of the National Health Act 1953.

Date                 30 November 2022                    

NIKOLAI TSYGANOV

Assistant Secretary (Acting)

Pricing and PBS Policy Branch

Technology Assessment and Access Division

Contents

1......... Name............................................................................................................................... 1

2......... Commencement............................................................................................................... 1

3......... Authority......................................................................................................................... 1

4......... Schedules......................................................................................................................... 1

Schedule 1—Amendments  2

National Health (Highly Specialised Drugs Program) Special Arrangement 2021
(PB 27 of 2021)   2

  1. Name

(1)This instrument is the National Health (Highly Specialised Drugs Program) Special Arrangement Amendment (December Update) Instrument 2022.

(2)This instrument may also be cited as PB 114 of 2022.

  1. Commencement

(1)Each provision of this instrument specified in column 1 of the table commences, or is taken to have commenced, in accordance with column 2 of the table. Any other statement in column 2 has effect according to its terms.

Commencement information
Column 1 Column 2 Column 3
Provisions Commencement Date/Details
1.  The whole of this instrument 1 December 2022 1 December 2022

Note:          This table relates only to the provisions of this instrument as originally made. It will not be amended to deal with any later amendments of this instrument.

(2)Any information in column 3 of the table is not part of this instrument. Information may be inserted in this column, or information in it may be edited, in any published version of this instrument.

  1. Authority

This instrument is made under subsection 100(2) of the National Health Act 1953.

  1. Schedules

Each instrument that is specified in a Schedule to this instrument is amended or repealed as set out in the applicable items in the Schedule concerned, and any other item in a Schedule to this instrument has effect according to its terms.

Schedule 1—Amendments

National Health (Highly Specialised Drugs Program) Special Arrangement 2021 (PB 27 of 2021)

  1. Part 1, Division 1, Section 6, definition for “CAR drug”

substitute:

CAR drug (short for Complex Authority Required drug) means any of the following highly specialised drugs:

(a)      abatacept;

(b)      adalimumab;

(c)      ambrisentan;

(d)      azacitidine;

(e)      benralizumab;

(f)      bosentan;

(g)      burosumab;     

(h)      dupilumab;

(i)       eculizumab;

(j)       elexacaftor with tezacaftor and with ivacaftor, and ivacaftor;

(k)      eltrombopag;

(l)       epoprostenol;

(m)     etanercept;

(n)      iloprost;

(o)      infliximab;

(p)      ivacaftor;

(q)      lenalidomide;

(r)      lumacaftor with ivacaftor;

(s)      macitentan;

(t)       mepolizumab;

(u)      midostaurin;

(v)      nusinersen;

(w)     omalizumab;

(x)      onasemnogene abeparvovec;

(y)      pasireotide;

(z)      pegcetacoplan;

(aa)    pegvisomant;

(bb)    pomalidomide;

(cc)    ravulizumab;

(dd)    riociguat;

(ee)    risdiplam;

(ff)     romiplostim;

(gg)    selexipag;

(hh)    sildenafil;

(ii)      tadalafil;

(jj)      teduglutide;

(kk)    tezacaftor with ivacaftor and ivacaftor;

(ll)      tocilizumab;

(mm)  ustekinumab;

(nn)    vedolizumab.

  1. Schedule 1, entry for Ambrisentan in each of the forms: Tablet 5 mg; and Tablet 10 mg

(a)omit from the column headed “Circumstances” (all instances): C11312 C11313 C11314 C12433 C12446 C12447 C12460

(b)insert in numerical order in the column headed “Circumstances” (all instances): C13496 C13497 C13499 C13500 C13575 C13576 C13580 C13582

  1. Schedule 1, entry for Benralizumab

omit:

Injection 30 mg in 1 mL single dose pre‑filled syringe Injection Fasenra C11841 C11842 C11892 C11893 See Schedule 2 See Schedule 2

  1. Schedule 1, entry for Bosentan in the form Tablet 62.5 mg (as monohydrate)

(a)omit from the column headed “Circumstances” (all instances): C11312 C11313 C11314 C12406 C12423

(b)omit from the column headed “Circumstances” (all instances): C12427 C12458

(c)insert in numerical order in the column headed “Circumstances” (all instances): C13495 C13496 C13497 C13499 C13571 C13580 C13582 C13632

  1. Schedule 1, entry for Bosentan in the form Tablet 125 mg (as monohydrate)

(a)omit from the column headed “Circumstances” (all instances): C11312 C11313 C11314 C12406 C12423 C12427 C12458

(b)insert in numerical order in the column headed “Circumstances” (all instances): C13495 C13496 C13497 C13499 C13571 C13580 C13582 C13632

  1. Schedule 1, entry for Eculizumab

(a)omit from the column headed “Circumstances”: C12944 C12945 C12953 C12954 C12955 C12964 C12969

(b)insert in numerical order in the column headed “Circumstances”: C13458 C13459 C13461 C13464 C13560 C13619 C13660 C13661 C13684

  1. Schedule 1, entry for Epoprostenol

substitute:

Epoprostenol Powder for I.V. infusion 500 micrograms (as sodium) Injection EPOPROSTENOL SUN C13491 C13492 C13505 C13506 C13510 C13512 C13577 C13634 See Schedule 2 See Schedule 2
Veletri C13491 C13492 C13505 C13506 C13510 C13512 C13577 C13634 See Schedule 2 See Schedule 2
Powder for I.V. infusion 500 micrograms (as sodium) with 2 vials diluent 50 mL Injection Flolan C13491 C13492 C13505 C13506 C13510 C13512 C13577 C13634 See Schedule 2 See Schedule 2
Powder for I.V. infusion 1.5 mg (as sodium) Injection EPOPROSTENOL SUN C13491 C13492 C13505 C13506 C13510 C13512 C13577 C13634 See Schedule 2 See Schedule 2
Veletri C13491 C13492 C13505 C13506 C13510 C13512 C13577 C13634 See Schedule 2 See Schedule 2
Powder for I.V. infusion 1.5 mg (as sodium) with 2 vials diluent 50 mL Injection Flolan C13491 C13492 C13505 C13506 C13510 C13512 C13577 C13634 See Schedule 2 See Schedule 2

  1. Schedule 1, entry for Iloprost

omit from the column headed “Circumstances”: C10229 C11322 C11323 C11325 C11343 C11345 C11356 C11365   substitute: C13491 C13492 C13505 C13506 C13510 C13577 C13631 C13634

  1. Schedule 1, entry for Infliximab

substitute:

Infliximab Powder for I.V. infusion 100 mg Injection Inflectra C4524 C7777 C8296 C8646 C8745 C8844 C8881 C8883 C8886 C8940 C8941 C8962 C9065 C9067 C9068 C9111 C9188 C9400 C9402 C9472 C9481 C9487 C9559 C9584 C9587 C9602 C9621 C9632 C9668 C9669 C9677 C9719 C9721 C9732 C9751 C9754 C9775 C9779 C9783 C9787 C9803 C11158 C12003 C12004 C12025 C12039 C12042 C12043 C12049 C12051 C12058 C12059 C12063 C12067 C12069 C12074 C12075 C12313 C12361 C12362 C13518 C13522 C13526 C13529 C13584 C13586 C13587 C13589 C13590 C13591 C13592 C13639 C13640 C13641 C13679 C13689 C13691 C13692 C13702 C13705 C13706 C13714 C13715 C13719 See Schedule 2 See Schedule 2
Remicade C4524 C7777 C8296 C8646 C8745 C8881 C8883 C8886 C8941 C8962 C9065 C9067 C9068 C9111 C9400 C9402 C9487 C9559 C9587 C9632 C9669 C9677 C9719 C9721 C9751 C9754 C9779 C9783 C9803 C11158 C12003 C12004 C12025 C12039 C12043 C12049 C12058 C12059 C12063 C12313 C12361 C12362 C13518 C13522 C13526 C13529 C13584 C13586 C13587 C13589 C13590 C13591 C13592 C13639 C13640 C13641 C13679 C13689 C13691 C13692 C13702 C13705 C13706 C13714 C13715 C13719 See Schedule 2 See Schedule 2
Renflexis C4524 C7777 C8296 C8646 C8745 C8844 C8881 C8883 C8886 C8940 C8941 C8962 C9065 C9067 C9068 C9111 C9188 C9400 C9402 C9472 C9481 C9487 C9559 C9584 C9587 C9602 C9621 C9632 C9668 C9669 C9677 C9719 C9721 C9732 C9751 C9754 C9775 C9779 C9783 C9787 C9803 C11158 C12003 C12004 C12025 C12039 C12042 C12043 C12049 C12051 C12058 C12059 C12063 C12067 C12069 C12074 C12075 C12313 C12361 C12362 C13518 C13522 C13526 C13529 C13584 C13586 C13587 C13589 C13590 C13591 C13592 C13639 C13640 C13641 C13679 C13689 C13691 C13692 C13702 C13705 C13706 C13714 C13715 C13719 See Schedule 2 See Schedule 2

  1. Schedule 1, entry for Macitentan

(a)omit from the column headed “Circumstances”: C11312 C11313 C11314 C12402 C12403 C12420 C12463

(b)insert in numerical order in the column headed “Circumstances”: C13496 C13497 C13499 C13500 C13575 C13576 C13580 C13582

  1. Schedule 1, entry for Mycophenolic acid in the form Tablet containing mycophenolate mofetil 500 mg

insert in the columns in the order indicated, and in alphabetical order for the column headed “Brand”:

Noumed Mycophenolate C5554 C5795 C9691 C9693 300 5

  1. Schedule 1, entry for Natalizumab

omit from the column headed “Circumstances”: C9744 C9818                   substitute: C13625 C13718

  1. Schedule 1, after entry for Pasireotide in the form Injection (modified release) 60 mg (as embonate), vial and diluent syringe

insert:

Pegcetacoplan Solution for subcutaneous infusion 1,080 mg in 20 mL Injection Empaveli C13616 C13655 C13658 C13710 C13743 See Schedule 2 See Schedule 2

  1. Schedule 1, entry for Ravulizumab in each of the forms: Solution concentrate for I.V. infusion 300 mg in 3 mL; and Solution concentrate for I.V. infusion 1,100 mg in 11 mL

omit from the column headed “Circumstances”: C12950 C12958 C12959 C12967 C12970 C12973             substitute: C13456 C13459 C13465 C13466 C13614 C13620 C13695

  1. Schedule 1, entry for Riociguat in each of the forms: Tablet 500 micrograms; Tablet 1 mg; Tablet 1.5 mg; Tablet 2 mg; and Tablet 2.5 mg

(a)omit from the column headed “Circumstances”: C10231 C10243 C10245

(b)insert in numerical order in the column headed “Circumstances”: C13502 C13514 C13515

  1. Schedule 1, entry for Sildenafil

(a)omit from the column headed “Circumstances” (all instances): C11319 C11338 C11350 C12430 C12431 C12441 C12443 C12456

(b)insert in numerical order in the column headed “Circumstances” (all instances): C13482 C13484 C13569 C13570 C13572 C13573 C13629 C13671

  1. Schedule 1, entry for Tadalafil

(a)omit from the column headed “Circumstances” (all instances): C11319 C11338 C11350 C12430 C12431 C12441 C12443 C12456

(b)insert in numerical order in the column headed “Circumstances” (all instances): C13482 C13484 C13569 C13570 C13572 C13573 C13629 C13671

  1. Schedule 1, entry for Valganciclovir in the form Tablet 450 mg (as hydrochloride)

insert in the columns in the order indicated, and in alphabetical order for the column headed “Brand”:

Valganciclovir Viatris C4980 C4989 C9316 120 5

  1. Schedule 2, entry for Ambrisentan

substitute:

Ambrisentan C11229 C13496 C13497 C13499 C13500 C13575 C13576 C13580 C13582 Sufficient for treatment for 1 month 5

  1. Schedule 2, entry for Bosentan

substitute:

Bosentan C11229 C13495 C13496 C13497 C13499 C13571 C13580 C13582 C13632 Sufficient for treatment for 1 month 5
C12425 Sufficient for treatment for 1 month 0

  1. Schedule 2, entry for Eculizumab

substitute:

Eculizumab C6626 1 Sufficient for 4 weeks of treatment
C6642 1 4
C6668 C6686 C6687 C6688 1 5
C6637 1 6
C13461 1 0
C13464 C13619 C13660 C13661 C13684 6 5
C13458 C13459 C13560 8 0

  1. Schedule 2, entry for Epoprostenol

substitute:

Epoprostenol C13491 C13492 C13505 C13506 C13510 C13512 C13577 C13634 Sufficient for treatment for 1 month 5

  1. Schedule 2, entry for Iloprost

substitute:

Iloprost C13491 C13492 C13505 C13506 C13510 C13577 C13631 C13634 Sufficient for treatment for 1 month 5

  1. Schedule 2, entry for Infliximab

substitute:

Infliximab C8886 C9111 C9400 C9402 C9487 C9559 C9587 C11158 C13518 C13529 C13584 C13586 C13587 C13589 C13590 C13591 C13592 C13640 C13679 C13689 C13692 C13705 C13706 C13715 C13719 1 dose of 5 mg per kg of patient weight 3
C8646 C12039 C12361 C13522 C13714 1 dose of 3 mg per kg of patient weight 3
C8745 C8844 C8940 C9188 C9472 C9481 C9584 C9602 C9621 C9668 C12004 C12058 C12067 C12075 C12362 1 dose of 3 mg per kg of patient weight 2
C7777 C8296 C8881 C8883 C8941 C8962 C9065 C9067 C9068 C9669 C9677 C9719 C9721 C9732 C9751 C9754 C9775 C9779 C9783 C9787 C9803 C12003 C12025 C12042 C12043 C12049 C12051 C12059 C12063 C12069 C12074 C12313 C13526 C13639 C13641 C13691 C13702 1 dose of 5 mg per kg of patient weight 2
C4524 C9632 5 vials 1

  1. Schedule 2, entry for Macitentan

substitute:

Macitentan C11229 C13496 C13497 C13499 C13500 C13575 C13576 C13580 C13582 Sufficient for treatment for 1 month 5

  1. Schedule 2, after entry for Pasireotide

insert:

Pegcetacoplan C13655 C13710 Sufficient for treatment for 4 weeks 0
C13616 C13658 C13743 Sufficient for treatment for 4 weeks 5

  1. Schedule 2, entry for Ravulizumab

substitute:

Ravulizumab C13456 C13459 C13614 C13620 1 dose 0
C13465 C13466 C13695 1 dose 2

  1. Schedule 2, entry for Riociguat

substitute:

Riociguat C6664 Sufficient for treatment for 1 month 3
C13514 C13515 Sufficient for treatment for 1 month 4
C6645 C7629 C13502 Sufficient for treatment for 1 month 5

  1. Schedule 2, entry for Sildenafil

substitute:

Sildenafil C11229 C13482 C13484 C13569 C13570 C13572 C13573 C13629 C13671 Sufficient for treatment for 1 month 5

  1. Schedule 2, entry for Tadalafil

substitute:

Tadalafil C11229 C13482 C13484 C13569 C13570 C13572 C13573 C13629 C13671 Sufficient for treatment for 1 month 5

  1. Schedule 3, entry for Ambrisentan

substitute:

Ambrisentan C11229 Pulmonary arterial hypertension (PAH)
Triple therapy - Initial treatment or continuing treatment of triple combination therapy (including dual therapy in lieu of triple therapy) that includes selexipag
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) PBS-subsidised selexipag (referred to as 'triple therapy'); OR
The treatment must form part of dual combination therapy consisting of either: (i) PBS-subsidised selexipag with one endothelin receptor antagonist, (ii) PBS-subsidised selexipag with one phosphodiesterase-5 inhibitor, as triple combination therapy with selexipag-an endothelin receptor antagonist-a phoshodiesterase-5 inhibitor is not possible due to an intolerance/contraindication to the endothelin receptor antagonist class/phosphodiesterase-5 inhibitor class (referred to as 'dual therapy in lieu of triple therapy').
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
The authority application for selexipag must be approved prior to the authority application for this agent.
For the purposes of PBS subsidy, an endothelin receptor antagonist is one of: (a) ambrisentan, (b) bosentan, (c) macitentan; a phosphodiesterase-5 inhibitor is one of: (d) sildenafil, (e) tadalafil.
PBS-subsidy does not cover patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
PAH (WHO Group 1 pulmonary hypertension) is defined as follows:
(i) mean pulmonary artery pressure (mPAP) greater than or equal to 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) less than or equal to 15 mmHg; or
(ii) where a right heart catheter (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure (RVSP), assessed by echocardiography (ECHO), greater than 40 mmHg, with normal left ventricular function.
The results and date of the RHC, ECHO and 6 MWT as applicable must be included in the patient's medical record. Where a RHC cannot be performed on clinical grounds, the written confirmation of the reasons why must also be included in the patient's medical record.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Authority Required procedures
C13496 Pulmonary arterial hypertension (PAH)
Initial 1 - combination therapy (dual or triple therapy, excluding selexipag) in an untreated patient
Patient must not have received prior PBS-subsidised treatment with a pulmonary arterial hypertension (PAH) agent; AND
Patient must currently have WHO Functional Class III PAH or WHO Functional Class IV PAH; AND
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one prostanoid; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid; triple combination therapy is treating a patient with class IV PAH.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that the test results are of a recency that the PAH physician making this authority application is satisfied that the diagnosis of PAH is current.
(5) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The test results must not be more than 6 months old at the time of application. 		Compliance with Written Authority Required procedures
C13497 Pulmonary arterial hypertension (PAH)
Initial 3 - changing to this drug in combination therapy (dual or triple therapy)
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one prostanoid; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH; AND
Patient must be undergoing existing PBS-subsidised combination therapy with at least this drug in the combination changing; combination therapy is not to commence through this Treatment phase listing. 		Compliance with Authority Required procedures
C13499 Pulmonary arterial hypertension (PAH)
Continuing treatment of combination therapy (dual or triple therapy, excluding selexipag)
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one prostanoid; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH; AND
Patient must be undergoing continuing treatment of existing PBS-subsidised combination therapy (dual/triple therapy, excluding selexipag), where this drug in the combination remains unchanged from the previous authority application. 		Compliance with Authority Required procedures
C13500 Pulmonary arterial hypertension (PAH)
Initial 1 (new patients)
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
Patient must not have received prior PBS-subsidised treatment with a pulmonary arterial hypertension (PAH) agent; AND
Patient must have WHO Functional Class II PAH, or WHO Functional Class III PAH, or WHO Functional Class IV PAH; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that the test results are of a recency that the PAH physician making this authority application is satisfied that the diagnosis of PAH is current.
(5) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The test results must not be more than 6 months old at the time of application.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Written Authority Required procedures
C13575 Pulmonary arterial hypertension (PAH)
Continuing treatment
Patient must have received their most recent course of PBS-subsidised treatment with this PAH agent for this condition; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
PAH agents are not PBS-subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Authority Required procedures
C13576 Pulmonary arterial hypertension (PAH)
Initial 2 (change)
Patient must have documented WHO Functional Class II PAH, or WHO Functional Class III PAH, or WHO Functional Class IV PAH; AND
Patient must have had their most recent course of PBS-subsidised treatment for this condition with a PAH agent other than this agent; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
PAH agents are not PBS-subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
Swapping between PAH agents: Patients can access PAH agents through the PBS according to the relevant restrictions. Once these patients are approved initial treatment (monotherapy) with 1 of these 8 drugs, they may swap between PAH agents at any time without having to re-qualify for treatment with the alternate agent. This means that patients may commence treatment with the alternate agent, subject to that agent's restriction, irrespective of the severity of their disease at the time the application to swap therapy is submitted.
Applications to swap between the 8 PAH agents must be made under the relevant initial treatment (monotherapy) restriction.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Authority Required procedures
C13580 Pulmonary arterial hypertension (PAH)
Transitioning from non-PBS to PBS-subsidised supply of combination therapy (dual or triple therapy, excluding selexipag) - Grandfather arrangements where each drug has not been a PBS benefit
Patient must have been receiving, prior to 1 December 2022, non-PBS-subsidised dual therapy consisting of one endothelin receptor antagonist with one prostanoid, where each drug was not a PBS benefit; this authority application is to continue such combination therapy; OR
Patient must have been receiving, prior to 1 December 2022, non-PBS-subsidised triple therapy consisting of one endothelin receptor antagonist, one prostanoid, one phosphodiesterase-5 inhibitor, where each drug was not a PBS benefit; this authority application is to continue such combination therapy; AND
The condition must have, prior to the time non-PBS combination therapy was initiated, progressed to at least Class III PAH despite treatment with at least one drug from the drug classes mentioned above; OR
The condition must have, at the time non-PBS combination therapy was initiated, been both: (i) classed as at least Class III PAH, (ii) untreated with any drug from the drug classes mentioned above.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted. 		Compliance with Authority Required procedures
C13582 Pulmonary arterial hypertension (PAH)
Initial 2 - starting combination therapy (dual or triple therapy, excluding selexipag) in a treated patient where a diagnosis of pulmonary arterial hypertension is established through a prior PBS authority application
Patient must currently have WHO Functional Class III PAH or WHO Functional Class IV PAH; AND
Patient must have failed to achieve/maintain WHO Functional Class II status with at least one of the following PBS-subsidised therapies: (i) endothelin receptor antagonist monotherapy, (ii) phosphodiesterase-5 inhibitor monotherapy, (iii) prostanoid monotherapy; AND
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one prostanoid; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid; triple combination therapy is treating a patient in whom monotherapy/dual combination therapy has been inadequate.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH. 		Compliance with Authority Required procedures

  1. Schedule 3, entry for Bosentan

substitute:

Bosentan C11229 Pulmonary arterial hypertension (PAH)
Triple therapy - Initial treatment or continuing treatment of triple combination therapy (including dual therapy in lieu of triple therapy) that includes selexipag
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) PBS-subsidised selexipag (referred to as 'triple therapy'); OR
The treatment must form part of dual combination therapy consisting of either: (i) PBS-subsidised selexipag with one endothelin receptor antagonist, (ii) PBS-subsidised selexipag with one phosphodiesterase-5 inhibitor, as triple combination therapy with selexipag-an endothelin receptor antagonist-a phoshodiesterase-5 inhibitor is not possible due to an intolerance/contraindication to the endothelin receptor antagonist class/phosphodiesterase-5 inhibitor class (referred to as 'dual therapy in lieu of triple therapy').
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
The authority application for selexipag must be approved prior to the authority application for this agent.
For the purposes of PBS subsidy, an endothelin receptor antagonist is one of: (a) ambrisentan, (b) bosentan, (c) macitentan; a phosphodiesterase-5 inhibitor is one of: (d) sildenafil, (e) tadalafil.
PBS-subsidy does not cover patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
PAH (WHO Group 1 pulmonary hypertension) is defined as follows:
(i) mean pulmonary artery pressure (mPAP) greater than or equal to 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) less than or equal to 15 mmHg; or
(ii) where a right heart catheter (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure (RVSP), assessed by echocardiography (ECHO), greater than 40 mmHg, with normal left ventricular function.
The results and date of the RHC, ECHO and 6 MWT as applicable must be included in the patient's medical record. Where a RHC cannot be performed on clinical grounds, the written confirmation of the reasons why must also be included in the patient's medical record.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Authority Required procedures
C12425 Pulmonary arterial hypertension (PAH)
Cessation of treatment (all patients)
Patient must be receiving PBS-subsidised treatment with this PAH agent; AND
The treatment must be for the purpose of gradual dose reduction prior to ceasing therapy.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment. Treatment beyond 1 month will not be approved. 		Compliance with Authority Required procedures
C13495 Pulmonary arterial hypertension (PAH)
Initial 2 (change)
Patient must have documented WHO Functional Class II PAH, or WHO Functional Class III PAH, or WHO Functional Class IV PAH; AND
Patient must have had their most recent course of PBS-subsidised treatment for this condition with a PAH agent other than this agent; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
PAH agents are not PBS-subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
Swapping between PAH agents: Patients can access PAH agents through the PBS according to the relevant restrictions. Once these patients are approved initial treatment (monotherapy) with 1 of these 8 drugs, they may swap between PAH agents at any time without having to re-qualify for treatment with the alternate agent. This means that patients may commence treatment with the alternate agent, subject to that agent's restriction, irrespective of the severity of their disease at the time the application to swap therapy is submitted.
Applications to swap between the 8 PAH agents must be made under the relevant initial treatment (monotherapy) restriction.
If patients will be taking 62.5mg for the first month then 125 mg, prescribers should request the first authority prescription of therapy with the 62.5 mg tablet strength, with the quantity for one month of treatment, based on the dosage recommendations in the TGA-approved Product Information and no repeats.
Prescribers should request the second authority prescription of therapy with the 125 mg tablet strengths, with a quantity for one month of treatment, based on the dosage recommendations in the TGA-approved Product Information, and a maximum of 4 repeats.
If patients will be taking 62.5mg for longer than 1 month, prescribers should request the first authority prescription of therapy with the 62.5 mg tablet strength, with the quantity for one month of treatment and a maximum of 5 repeats based on the dosage recommendations in the TGA-approved Product Information. 		Compliance with Authority Required procedures
C13496 Pulmonary arterial hypertension (PAH)
Initial 1 - combination therapy (dual or triple therapy, excluding selexipag) in an untreated patient
Patient must not have received prior PBS-subsidised treatment with a pulmonary arterial hypertension (PAH) agent; AND
Patient must currently have WHO Functional Class III PAH or WHO Functional Class IV PAH; AND
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one prostanoid; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid; triple combination therapy is treating a patient with class IV PAH.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that the test results are of a recency that the PAH physician making this authority application is satisfied that the diagnosis of PAH is current.
(5) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The test results must not be more than 6 months old at the time of application. 		Compliance with Written Authority Required procedures
C13497 Pulmonary arterial hypertension (PAH)
Initial 3 - changing to this drug in combination therapy (dual or triple therapy)
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one prostanoid; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH; AND
Patient must be undergoing existing PBS-subsidised combination therapy with at least this drug in the combination changing; combination therapy is not to commence through this Treatment phase listing. 		Compliance with Authority Required procedures
C13499 Pulmonary arterial hypertension (PAH)
Continuing treatment of combination therapy (dual or triple therapy, excluding selexipag)
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one prostanoid; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH; AND
Patient must be undergoing continuing treatment of existing PBS-subsidised combination therapy (dual/triple therapy, excluding selexipag), where this drug in the combination remains unchanged from the previous authority application. 		Compliance with Authority Required procedures
C13571 Pulmonary arterial hypertension (PAH)
Continuing treatment
Patient must have received their most recent course of PBS-subsidised treatment with this PAH agent for this condition; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
PAH agents are not PBS-subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Authority Required procedures
C13580 Pulmonary arterial hypertension (PAH)
Transitioning from non-PBS to PBS-subsidised supply of combination therapy (dual or triple therapy, excluding selexipag) - Grandfather arrangements where each drug has not been a PBS benefit
Patient must have been receiving, prior to 1 December 2022, non-PBS-subsidised dual therapy consisting of one endothelin receptor antagonist with one prostanoid, where each drug was not a PBS benefit; this authority application is to continue such combination therapy; OR
Patient must have been receiving, prior to 1 December 2022, non-PBS-subsidised triple therapy consisting of one endothelin receptor antagonist, one prostanoid, one phosphodiesterase-5 inhibitor, where each drug was not a PBS benefit; this authority application is to continue such combination therapy; AND
The condition must have, prior to the time non-PBS combination therapy was initiated, progressed to at least Class III PAH despite treatment with at least one drug from the drug classes mentioned above; OR
The condition must have, at the time non-PBS combination therapy was initiated, been both: (i) classed as at least Class III PAH, (ii) untreated with any drug from the drug classes mentioned above.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted. 		Compliance with Authority Required procedures
C13582 Pulmonary arterial hypertension (PAH)
Initial 2 - starting combination therapy (dual or triple therapy, excluding selexipag) in a treated patient where a diagnosis of pulmonary arterial hypertension is established through a prior PBS authority application
Patient must currently have WHO Functional Class III PAH or WHO Functional Class IV PAH; AND
Patient must have failed to achieve/maintain WHO Functional Class II status with at least one of the following PBS-subsidised therapies: (i) endothelin receptor antagonist monotherapy, (ii) phosphodiesterase-5 inhibitor monotherapy, (iii) prostanoid monotherapy; AND
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one prostanoid; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid; triple combination therapy is treating a patient in whom monotherapy/dual combination therapy has been inadequate.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH. 		Compliance with Authority Required procedures
C13632 Pulmonary arterial hypertension (PAH)
Initial 1 (new patients)
Patient must not have received prior PBS-subsidised treatment with a pulmonary arterial hypertension (PAH) agent; AND
Patient must have WHO Functional Class II PAH, or WHO Functional Class III PAH, or WHO Functional Class IV PAH; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that the test results are of a recency that the PAH physician making this authority application is satisfied that the diagnosis of PAH is current.
(5) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The test results must not be more than 6 months old at the time of application.
If patients will be taking 62.5mg for the first month then 125 mg, prescribers should request the first authority prescription of therapy with the 62.5 mg tablet strength, with the quantity for one month of treatment, based on the dosage recommendations in the TGA-approved Product Information and no repeats.
Prescribers should request the second authority prescription of therapy with the 125 mg tablet strengths, with a quantity for one month of treatment, based on the dosage recommendations in the TGA-approved Product Information, and a maximum of 4 repeats.
If patients will be taking 62.5mg for longer than 1 month, prescribers should request the first authority prescription of therapy with the 62.5 mg tablet strength, with the quantity for one month of treatment and a maximum of 5 repeats based on the dosage recommendations in the TGA-approved Product Information. 		Compliance with Written Authority Required procedures

  1. Schedule 3, entry for Burosumab

substitute:

Burosumab C13330 X-linked hypophosphataemia
Continuing treatment
Patient must have previously received PBS-subsidised treatment with this drug for this condition; AND
Patient must have achieved normalisation in serum phosphate levels; AND
Patient must have radiographical evidence of stabilisation/improvement in rickets in patients without growth plate fusion.
Must be treated by a medical practitioner identifying as at least one of the following specialists: (i) paediatric endocrinologist, (ii) paediatric nephrologist, (iii) endocrinologist, (iv) nephrologist.
Where adequate response to treatment with this drug cannot be demonstrated, the treating physician must confirm that continuing therapy has been determined to be clinically required by a second specialist physician with expertise in the treatment of X-linked hypophosphataemia.
At the time of authority application, medical practitioners must request the appropriate number of vials of appropriate strength(s) to provide sufficient drug, based on the weight of the patient, adequate for 4 weeks, according to the specified dosage in the approved Product Information (PI). A separate authority prescription form must be completed for each strength requested. Up to a maximum of 5 repeats will be authorised.
Confirmation of eligibility for treatment with diagnostic reports must be documented in the patient's medical records. 		Compliance with Authority Required procedures
C13377 X-linked hypophosphataemia
Initial treatment - New patient
Patient must have a documented confirmation of PHEX pathogenic variant; OR
Patient must have a confirmed diagnosis of X-linked hypophosphataemia demonstrated by the presence of all of the following: (i) a serum phosphate concentration below the age adjusted lower limit of normal; (ii) current or historical (for those with growth plate fusion) radiographic X-ray evidence of rickets; (iii) elevated (or inappropriately normal) serum or plasma FGF-23 levels of above the mean of the assay-specific reference range; (iv) renal phosphate wasting demonstrated by a ratio of tubular maximum reabsorption rate of phosphate to glomerular filtration rate (TmP/GFR) according to age specific normal ranges using the second morning urine void and paired serum sample measuring phosphate and creatinine.
Must be treated by a medical practitioner identifying as at least one of the following specialists: (i) paediatric endocrinologist, (ii) paediatric nephrologist, (iii) endocrinologist, (iv) nephrologist.
At the time of authority application, medical practitioners must request the appropriate number of vials of appropriate strength(s) to provide sufficient drug, based on the weight of the patient, adequate for 4 weeks, according to the specified dosage in the approved Product Information (PI). A separate authority prescription form must be completed for each strength requested. Up to a maximum of 5 repeats will be authorised.
Confirmation of eligibility for treatment with diagnostic reports must be documented in the patient's medical records. 		Compliance with Authority Required procedures
C13400 X-linked hypophosphataemia
Transitioning from non-PBS to PBS-subsidised supply - Grandfather arrangements
Patient must have received non-PBS-subsidised treatment with this drug for this condition prior to 1 November 2022; AND
Patient must have a documented confirmation of PHEX pathogenic variant; OR
Patient must have, prior to commencing non-PBS-subsidised supply, a confirmed diagnosis of X-linked hypophosphataemia demonstrated by the presence of all of the following: (i) a serum phosphate concentration below the age adjusted lower limit of normal; (ii) current or historical (for those with growth plate fusion) radiographic evidence of rickets; (iii) elevated (or inappropriately normal) serum or plasma FGF-23 levels of above the mean of the assay-specific reference range; (iv) renal phosphate wasting demonstrated by a ratio of tubular maximum reabsorption rate of phosphate to glomerular filtration rate (TmP/GFR) according to age specific normal ranges using the second morning urine void and paired serum sample measuring phosphate and creatinine; AND
Patient must have achieved normalisation in serum phosphate levels; AND
Patient must have radiographical evidence of stabilisation/improvement in rickets in patients without growth plate fusion.
Must be treated by a medical practitioner identifying as at least one of the following specialists: (i) paediatric endocrinologist, (ii) paediatric nephrologist, (iii) endocrinologist, (iv) nephrologist.
Where adequate response to treatment with this drug cannot be demonstrated, the treating physician must confirm that continuing therapy has been determined to be clinically required by a second specialist physician with expertise in the treatment of X-linked hypophosphataemia.
At the time of authority application, medical practitioners must request the appropriate number of vials of appropriate strength(s) to provide sufficient drug, based on the weight of the patient, adequate for 4 weeks, according to the specified dosage in the approved Product Information (PI). A separate authority prescription form must be completed for each strength requested. Up to a maximum of 5 repeats will be authorised.
Confirmation of eligibility for treatment with diagnostic reports must be documented in the patient's medical records. 		Compliance with Authority Required procedures

  1. Schedule 3, entry for Eculizumab

(a)omit:

C12944 Paroxysmal nocturnal haemoglobinuria (PNH)
First Continuing Treatment
Patient must have received PBS‑subsidised treatment with this drug for this condition under an 'Initial', 'Balance of Supply', or 'Grandfather' treatment criteria; AND
The treatment must not be in combination with ravulizumab.
Must be treated by a haematologist; OR
Must be treated by a non‑specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
At the time of the authority application, details (result and date of result) of the following monitoring requirements must be provided:
(i) Haemoglobin (g/L)
(ii) Platelets (x109/L)
(iii) White Cell Count (x109/L)
(iv) Reticulocytes (x109/L)
(v) Neutrophils (x109/L)
(vi) Granulocyte clone size (%)
(vii) Lactate Dehydrogenase (LDH) and the upper limit of normal (ULN) for the reporting laboratory
(viii) Multiple of LDH , ULN 		Compliance with Written Authority Required procedures
C12945 Paroxysmal nocturnal haemoglobinuria (PNH)
Grandfather 2 (transition from LSDP‑funded eculizumab)
Patient must have previously received eculizumab for the treatment of this condition funded under the Australian Government's Life Saving Drugs Program (LSDP); AND
Patient must have a diagnosis of PNH established by flow cytometry prior to commencing treatment with eculizumab; AND
Patient must have a PNH granulocyte clone size equal to or greater than 10% prior to commencing treatment with eculizumab; AND
Patient must have a raised lactate dehydrogenase value at least 1.5 times the upper limit of normal prior to commencing treatment with eculizumab; AND
Patient must have demonstrated clinical improvement or stabilisation of condition; AND
Patient must have experienced a thrombotic/embolic event which required anticoagulant therapy prior to commencing treatment with eculizumab; OR
Patient must have been transfused with at least 4 units of red blood cells in the last 12 months prior to commencing treatment with eculizumab; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 70 g/L in the absence of anaemia symptoms prior to commencing treatment with eculizumab; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 100 g/L in addition to having anaemia symptoms prior to commencing treatment with eculizumab; OR
Patient must have debilitating shortness of breath/chest pain resulting in limitation of normal activity (New York Heart Association Class III) and/or established diagnosis of pulmonary arterial hypertension, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; OR
Patient must have a history of renal insufficiency, demonstrated by an eGFR less than or equal to 60 mL/min/1.73m2, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; OR
Patient must have recurrent episodes of severe pain requiring hospitalisation and/or narcotic analgesia, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; AND
The treatment must not be in combination with ravulizumab.
Must be treated by a haematologist; OR
Must be treated by a non‑specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
At the time of the authority application, details (result and date of result) of the following monitoring requirements must be provided:
(i) Haemoglobin (g/L)
(ii) Platelets (x109/L)
(iii) White Cell Count (x109/L)
(iv) Reticulocytes (x109/L)
(v) Neutrophils (x109/L)
(vi) Granulocyte clone size (%)
(vii) Lactate Dehydrogenase (LDH) and the upper limit of normal (ULN) for the reporting laboratory
(viii) Multiple of LDH , ULN 		Compliance with Written Authority Required procedures
C12953 Paroxysmal nocturnal haemoglobinuria (PNH)
Initial treatment ‑ Initial 1 (new patient) induction doses
Patient must not have received prior treatment with this drug for this condition; AND
Patient must have a diagnosis of PNH established by flow cytometry; AND
Patient must have a PNH granulocyte clone size equal to or greater than 10%; AND
Patient must have a raised lactate dehydrogenase value at least 1.5 times the upper limit of normal; AND
Patient must have experienced a thrombotic/embolic event which required anticoagulant therapy; OR
Patient must have been transfused with at least 4 units of red blood cells in the last 12 months; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 70 g/L in the absence of anaemia symptoms; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 100 g/L in addition to having anaemia symptoms; OR
Patient must have debilitating shortness of breath/chest pain resulting in limitation of normal activity (New York Heart Association Class III) and/or established diagnosis of pulmonary arterial hypertension, where causes other than PNH have been excluded; OR
Patient must have a history of renal insufficiency, demonstrated by an eGFR less than or equal to 60 mL/min/1.73m2, where causes other than PNH have been excluded; OR
Patient must have recurrent episodes of severe pain requiring hospitalisation and/or narcotic analgesia, where causes other than PNH have been excluded; AND
The treatment must not be in combination with ravulizumab.
Must be treated by a haematologist; OR
Must be treated by a non‑specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
At the time of the authority application, details (result and date of result) of the following monitoring requirements must be provided:
(i) Haemoglobin (g/L)
(ii) Platelets (x109/L)
(iii) White Cell Count (x109/L)
(iv) Reticulocytes (x109/L)
(v) Neutrophils (x109/L)
(vi) Granulocyte clone size (%)
(vii) Lactate Dehydrogenase (LDH) and the upper limit of normal (ULN) for the reporting laboratory
(viii) Multiple of LDH , ULN 		Compliance with Written Authority Required procedures
C12954 Paroxysmal nocturnal haemoglobinuria (PNH)
Subsequent Continuing Treatment
Patient must have previously received PBS‑subsidised treatment with this drug for this condition under the 'First Continuing Treatment' or 'Switch' criteria; AND
Patient must have demonstrated clinical improvement or stabilisation of condition; AND
The treatment must not be in combination with ravulizumab.
Must be treated by a haematologist; OR
Must be treated by a non‑specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice). 		Compliance with Written Authority Required procedures
C12955 Paroxysmal nocturnal haemoglobinuria (PNH)
Balance of Supply (transition from non‑PBS‑subsidised treatment during induction phase)
Patient must have received non‑PBS‑subsidised eculizumab for this condition prior to 1 March 2022; AND
Patient must have received insufficient quantity to complete the induction treatment phase; AND
Patient must have a diagnosis of PNH established by flow cytometry prior to commencing treatment with eculizumab; AND
Patient must have a PNH granulocyte clone size equal to or greater than 10% prior to commencing treatment with eculizumab; AND
Patient must have a raised lactate dehydrogenase value at least 1.5 times the upper limit of normal prior to commencing treatment with eculizumab; AND
Patient must have experienced a thrombotic/embolic event which required anticoagulant therapy prior to commencing treatment with eculizumab; OR
Patient must have been transfused with at least 4 units of red blood cells in the last 12 months prior to commencing treatment with eculizumab; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 70 g/L in the absence of anaemia symptoms prior to commencing treatment with eculizumab; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 100 g/L in addition to having anaemia symptoms prior to commencing treatment with eculizumab; OR
Patient must have debilitating shortness of breath/chest pain resulting in limitation of normal activity (New York Heart Association Class III) and/or established diagnosis of pulmonary arterial hypertension, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; OR
Patient must have a history of renal insufficiency, demonstrated by an eGFR less than or equal to 60 mL/min/1.73m2, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; OR
Patient must have recurrent episodes of severe pain requiring hospitalisation and/or narcotic analgesia, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; AND
The treatment must not be in combination with ravulizumab.
Must be treated by a haematologist; OR
Must be treated by a non‑specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
At the time of the authority application, medical practitioners should request the appropriate number of vials to complete the induction treatment phase, as per the Product Information.
At the time of the authority application, details (result and date of result) of the following monitoring requirements must be provided:
(i) Haemoglobin (g/L)
(ii) Platelets (x109/L)
(iii) White Cell Count (x109/L)
(iv) Reticulocytes (x109/L)
(v) Neutrophils (x109/L)
(vi) Granulocyte clone size (%)
(vii) Lactate Dehydrogenase (LDH) and the upper limit of normal (ULN) for the reporting laboratory
(viii) Multiple of LDH , ULN 		Compliance with Written Authority Required procedures
C12964 Paroxysmal nocturnal haemoglobinuria (PNH)
Initial treatment ‑ Initial 2 (switching from PBS‑subsidised ravulizumab for pregnancy)
Patient must be planning pregnancy; OR
Patient must be pregnant; AND
Patient must have received PBS‑subsidised treatment with ravulizumab for this condition; AND
The treatment must not be in combination with ravulizumab.
Must be treated by a haematologist; OR
Must be treated by a non‑specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
Patient may qualify under this treatment phase more than once. In the event of miscarriage, patient may continue on eculizumab if patient is stable, and/or is planning a subsequent pregnancy. For continuing PBS‑subsidised treatment, a 'Switching' patient must proceed under the 'Subsequent Continuing Treatment' criteria. 		Compliance with Written Authority Required procedures
C12969 Paroxysmal nocturnal haemoglobinuria (PNH)
Grandfather 1 (transition from non‑PBS‑subsidised treatment) ‑ maintenance phase
Patient must have received non‑PBS‑subsidised eculizumab for this condition prior to 1 March 2022; AND
Patient must have a diagnosis of PNH established by flow cytometry prior to commencing treatment with eculizumab; AND
Patient must have a PNH granulocyte clone size equal to or greater than 10% prior to commencing treatment with eculizumab; AND
Patient must have a raised lactate dehydrogenase value at least 1.5 times the upper limit of normal prior to commencing treatment with eculizumab; AND
Patient must have demonstrated clinical improvement or stabilisation of condition; AND
Patient must have experienced a thrombotic/embolic event which required anticoagulant therapy prior to commencing treatment with eculizumab; OR
Patient must have been transfused with at least 4 units of red blood cells in the last 12 months prior to commencing treatment with eculizumab; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 70 g/L in the absence of anaemia symptoms prior to commencing treatment with eculizumab; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 100 g/L in addition to having anaemia symptoms prior to commencing treatment with eculizumab; OR
Patient must have debilitating shortness of breath/chest pain resulting in limitation of normal activity (New York Heart Association Class III) and/or established diagnosis of pulmonary arterial hypertension, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; OR
Patient must have a history of renal insufficiency, demonstrated by an eGFR less than or equal to 60 mL/min/1.73m2, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; OR
Patient must have recurrent episodes of severe pain requiring hospitalisation and/or narcotic analgesia, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; AND
The treatment must not be in combination with ravulizumab.
Must be treated by a haematologist; OR
Must be treated by a non‑specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
At the time of the authority application, details (result and date of result) of the following monitoring requirements must be provided:
(i) Haemoglobin (g/L)
(ii) Platelets (x109/L)
(iii) White Cell Count (x109/L)
(iv) Reticulocytes (x109/L)
(v) Neutrophils (x109/L)
(vi) Granulocyte clone size (%)
(vii) Lactate Dehydrogenase (LDH) and the upper limit of normal (ULN) for the reporting laboratory
(viii) Multiple of LDH , ULN 		Compliance with Written Authority Required procedures

(b)insert in numerical order after existing text:

C13458 Paroxysmal nocturnal haemoglobinuria (PNH)
Initial treatment - (initial 3) switching from PBS-subsidised pegcetacoplan for pregnancy (induction doses)
Patient must be planning pregnancy; OR
Patient must be pregnant; AND
Patient must have received PBS-subsidised treatment with pegcetacoplan for this condition; AND
The treatment must not be in combination with any of (i) ravulizumab, (ii) pegcetacoplan.
Must be treated by a haematologist; OR
Must be treated by a non-specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
Patient may qualify under this treatment phase more than once. In the event of miscarriage, patient may continue on eculizumab if patient is stable, and/or is planning a subsequent pregnancy. For continuing PBS-subsidised treatment, a 'Switching' patient must proceed under the 'Subsequent Continuing Treatment' criteria. 		Compliance with Written Authority Required procedures
C13459 Paroxysmal nocturnal haemoglobinuria (PNH)
Return from PBS-subsidised pegcetacoplan - induction doses
Patient must have received PBS-subsidised treatment with at least one Complement 5 (C5) inhibitor for this condition; AND
Patient must have received PBS-subsidised treatment with pegcetacoplan for this condition; AND
Patient must have developed resistance or intolerance to pegcetacoplan; AND
The treatment must not be in combination with any of (i) another Complement 5 (C5) inhibitor, (ii) pegcetacoplan.
Must be treated by a haematologist; OR
Must be treated by a non-specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
For continuing PBS-subsidised treatment with this drug, a 'Returning' patient must proceed under the 'Subsequent Continuing Treatment' criteria. 		Compliance with Written Authority Required procedures
C13461 Paroxysmal nocturnal haemoglobinuria (PNH)
Balance of Supply (transition from non-PBS-subsidised treatment during induction phase)
Patient must have received non-PBS-subsidised eculizumab for this condition prior to 1 March 2022; AND
Patient must have received insufficient quantity to complete the induction treatment phase; AND
Patient must have a diagnosis of PNH established by flow cytometry prior to commencing treatment with eculizumab; AND
Patient must have a PNH granulocyte clone size equal to or greater than 10% prior to commencing treatment with eculizumab; AND
Patient must have a raised lactate dehydrogenase value at least 1.5 times the upper limit of normal prior to commencing treatment with eculizumab; AND
Patient must have experienced a thrombotic/embolic event which required anticoagulant therapy prior to commencing treatment with eculizumab; OR
Patient must have been transfused with at least 4 units of red blood cells in the last 12 months prior to commencing treatment with eculizumab; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 70 g/L in the absence of anaemia symptoms prior to commencing treatment with eculizumab; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 100 g/L in addition to having anaemia symptoms prior to commencing treatment with eculizumab; OR
Patient must have debilitating shortness of breath/chest pain resulting in limitation of normal activity (New York Heart Association Class III) and/or established diagnosis of pulmonary arterial hypertension, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; OR
Patient must have a history of renal insufficiency, demonstrated by an eGFR less than or equal to 60 mL/min/1.73m2, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; OR
Patient must have recurrent episodes of severe pain requiring hospitalisation and/or narcotic analgesia, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; AND
The treatment must not be in combination with any of (i) another Complement 5 (C5) inhibitor, (ii) pegcetacoplan.
Must be treated by a haematologist; OR
Must be treated by a non-specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
At the time of the authority application, medical practitioners should request the appropriate number of vials to complete the induction treatment phase, as per the Product Information.
At the time of the authority application, details (result and date of result) of the following monitoring requirements must be provided:
(i) Haemoglobin (g/L)
(ii) Platelets (x109/L)
(iii) White Cell Count (x109/L)
(iv) Reticulocytes (x109/L)
(v) Neutrophils (x109/L)
(vi) Granulocyte clone size (%)
(vii) Lactate Dehydrogenase (LDH)
(viii) the upper limit of normal (ULN) for LDH as quoted by the reporting laboratory
(ix) the LDH:ULN ratio (in figures, rounded to one decimal place) must be at least 1.5 		Compliance with Written Authority Required procedures
C13464 Paroxysmal nocturnal haemoglobinuria (PNH)
Grandfather 1 (transition from non-PBS-subsidised treatment) - maintenance phase
Patient must have received non-PBS-subsidised eculizumab for this condition prior to 1 March 2022; AND
Patient must have a diagnosis of PNH established by flow cytometry prior to commencing treatment with eculizumab; AND
Patient must have a PNH granulocyte clone size equal to or greater than 10% prior to commencing treatment with eculizumab; AND
Patient must have a raised lactate dehydrogenase value at least 1.5 times the upper limit of normal prior to commencing treatment with eculizumab; AND
Patient must have experienced clinical improvement as a result of treatment with this drug; OR
Patient must have experienced a stabilisation of the condition as a result of treatment with this drug; AND
Patient must have experienced a thrombotic/embolic event which required anticoagulant therapy prior to commencing treatment with eculizumab; OR
Patient must have been transfused with at least 4 units of red blood cells in the last 12 months prior to commencing treatment with eculizumab; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 70 g/L in the absence of anaemia symptoms prior to commencing treatment with eculizumab; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 100 g/L in addition to having anaemia symptoms prior to commencing treatment with eculizumab; OR
Patient must have debilitating shortness of breath/chest pain resulting in limitation of normal activity (New York Heart Association Class III) and/or established diagnosis of pulmonary arterial hypertension, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; OR
Patient must have a history of renal insufficiency, demonstrated by an eGFR less than or equal to 60 mL/min/1.73m2, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; OR
Patient must have recurrent episodes of severe pain requiring hospitalisation and/or narcotic analgesia, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; AND
The treatment must not be in combination with any of (i) another Complement 5 (C5) inhibitor, (ii) pegcetacoplan.
Must be treated by a haematologist; OR
Must be treated by a non-specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
At the time of the authority application, details (result and date of result) of the following monitoring requirements must be provided:
(i) Haemoglobin (g/L)
(ii) Platelets (x109/L)
(iii) White Cell Count (x109/L)
(iv) Reticulocytes (x109/L)
(v) Neutrophils (x109/L)
(vi) Granulocyte clone size (%)
(vii) Lactate Dehydrogenase (LDH)
(viii) the upper limit of normal (ULN) for LDH as quoted by the reporting laboratory
(ix) the LDH:ULN ratio (in figures, rounded to one decimal place) must be at least 1.5 		Compliance with Written Authority Required procedures
C13560 Paroxysmal nocturnal haemoglobinuria (PNH)
Initial treatment - initial 1 (new patient) induction doses
Patient must not have received prior treatment with this drug for this condition; AND
Patient must have a diagnosis of PNH established by flow cytometry; AND
Patient must have a PNH granulocyte clone size equal to or greater than 10%; AND
Patient must have a raised lactate dehydrogenase value at least 1.5 times the upper limit of normal; AND
Patient must have experienced a thrombotic/embolic event which required anticoagulant therapy; OR
Patient must have been transfused with at least 4 units of red blood cells in the last 12 months; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 70 g/L in the absence of anaemia symptoms; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 100 g/L in addition to having anaemia symptoms; OR
Patient must have debilitating shortness of breath/chest pain resulting in limitation of normal activity (New York Heart Association Class III) and/or established diagnosis of pulmonary arterial hypertension, where causes other than PNH have been excluded; OR
Patient must have a history of renal insufficiency, demonstrated by an eGFR less than or equal to 60 mL/min/1.73m2, where causes other than PNH have been excluded; OR
Patient must have recurrent episodes of severe pain requiring hospitalisation and/or narcotic analgesia, where causes other than PNH have been excluded; AND
The treatment must not be in combination with any of (i) another Complement 5 (C5) inhibitor, (ii) pegcetacoplan.
Must be treated by a haematologist; OR
Must be treated by a non-specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
At the time of the authority application, details (result and date of result) of the following monitoring requirements must be provided:
(i) Haemoglobin (g/L)
(ii) Platelets (x109/L)
(iii) White Cell Count (x109/L)
(iv) Reticulocytes (x109/L)
(v) Neutrophils (x109/L)
(vi) Granulocyte clone size (%)
(vii) Lactate Dehydrogenase (LDH)
(viii) the upper limit of normal (ULN) for LDH as quoted by the reporting laboratory
(ix) the LDH:ULN ratio (in figures, rounded to one decimal place) must be at least 1.5 		Compliance with Written Authority Required procedures
C13619 Paroxysmal nocturnal haemoglobinuria (PNH)
First Continuing Treatment
Patient must have received PBS-subsidised treatment with this drug for this condition under an 'Initial', 'Balance of Supply', or 'Grandfather' treatment criteria; AND
The treatment must not be in combination with any of (i) another Complement 5 (C5) inhibitor, (ii) pegcetacoplan.
Must be treated by a haematologist; OR
Must be treated by a non-specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
At the time of the authority application, details (result and date of result) of the following monitoring requirements must be provided:
(i) Haemoglobin (g/L)
(ii) Platelets (x109/L)
(iii) White Cell Count (x109/L)
(iv) Reticulocytes (x109/L)
(v) Neutrophils (x109/L)
(vi) Granulocyte clone size (%)
(vii) Lactate Dehydrogenase (LDH)
(viii) the upper limit of normal (ULN) for LDH as quoted by the reporting laboratory
(ix) the LDH:ULN ratio (in figures, rounded to one decimal place) must be at least 1.5 		Compliance with Written Authority Required procedures
C13660 Paroxysmal nocturnal haemoglobinuria (PNH)
Grandfather 2 (transition from LSDP-funded eculizumab)
Patient must have previously received eculizumab for the treatment of this condition funded under the Australian Government's Life Saving Drugs Program (LSDP); AND
Patient must have a diagnosis of PNH established by flow cytometry prior to commencing treatment with eculizumab; AND
Patient must have a PNH granulocyte clone size equal to or greater than 10% prior to commencing treatment with eculizumab; AND
Patient must have a raised lactate dehydrogenase value at least 1.5 times the upper limit of normal prior to commencing treatment with eculizumab; AND
Patient must have experienced clinical improvement as a result of treatment with this drug; OR
Patient must have experienced a stabilisation of the condition as a result of treatment with this drug; AND
Patient must have experienced a thrombotic/embolic event which required anticoagulant therapy prior to commencing treatment with eculizumab; OR
Patient must have been transfused with at least 4 units of red blood cells in the last 12 months prior to commencing treatment with eculizumab; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 70 g/L in the absence of anaemia symptoms prior to commencing treatment with eculizumab; OR
Patient must have chronic/recurrent anaemia, where causes other than haemolysis have been excluded, together with multiple haemoglobin measurements not exceeding 100 g/L in addition to having anaemia symptoms prior to commencing treatment with eculizumab; OR
Patient must have debilitating shortness of breath/chest pain resulting in limitation of normal activity (New York Heart Association Class III) and/or established diagnosis of pulmonary arterial hypertension, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; OR
Patient must have a history of renal insufficiency, demonstrated by an eGFR less than or equal to 60 mL/min/1.73m2, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; OR
Patient must have recurrent episodes of severe pain requiring hospitalisation and/or narcotic analgesia, where causes other than PNH have been excluded prior to commencing treatment with eculizumab; AND
The treatment must not be in combination with any of (i) another Complement 5 (C5) inhibitor, (ii) pegcetacoplan.
Must be treated by a haematologist; OR
Must be treated by a non-specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
At the time of the authority application, details (result and date of result) of the following monitoring requirements must be provided:
(i) Haemoglobin (g/L)
(ii) Platelets (x109/L)
(iii) White Cell Count (x109/L)
(iv) Reticulocytes (x109/L)
(v) Neutrophils (x109/L)
(vi) Granulocyte clone size (%)
(vii) Lactate Dehydrogenase (LDH)
(viii) the upper limit of normal (ULN) for LDH as quoted by the reporting laboratory
(ix) the LDH:ULN ratio (in figures, rounded to one decimal place) must be at least 1.5 		Compliance with Written Authority Required procedures
C13661 Paroxysmal nocturnal haemoglobinuria (PNH)
Subsequent Continuing Treatment
Patient must have previously received PBS-subsidised treatment with this drug for this condition under the 'First Continuing Treatment' or 'Switch' criteria; AND
Patient must have experienced clinical improvement as a result of treatment with this drug; OR
Patient must have experienced a stabilisation of the condition as a result of treatment with this drug; AND
The treatment must not be in combination with any of (i) another Complement 5 (C5) inhibitor, (ii) pegcetacoplan.
Must be treated by a haematologist; OR
Must be treated by a non-specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice). 		Compliance with Written Authority Required procedures
C13684 Paroxysmal nocturnal haemoglobinuria (PNH)
Initial treatment - Initial 2 (switching from PBS-subsidised ravulizumab for pregnancy)
Patient must be planning pregnancy; OR
Patient must be pregnant; AND
Patient must have received PBS-subsidised treatment with ravulizumab for this condition; AND
The treatment must not be in combination with any of (i) another Complement 5 (C5) inhibitor, (ii) pegcetacoplan.
Must be treated by a haematologist; OR
Must be treated by a non-specialist medical physician who has consulted a haematologist on the patient's drug treatment details.
The authority application must be made in writing and must include:
(1) a completed authority prescription form; and
(2) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
Patient may qualify under this treatment phase more than once. In the event of miscarriage, patient may continue on eculizumab if patient is stable, and/or is planning a subsequent pregnancy. For continuing PBS-subsidised treatment, a 'Switching' patient must proceed under the 'Subsequent Continuing Treatment' criteria. 		Compliance with Written Authority Required procedures

  1. Schedule 3, entry for Epoprostenol

substitute:

Epoprostenol C13491 Pulmonary arterial hypertension (PAH)
Initial 2 (change)
Patient must have documented WHO Functional Class III PAH or WHO Functional Class IV PAH; AND
Patient must have had their most recent course of PBS-subsidised treatment for this condition with a PAH agent other than this agent; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
PAH agents are not PBS-subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
Swapping between PAH agents: Patients can access PAH agents through the PBS according to the relevant restrictions. Once these patients are approved initial treatment (monotherapy) with 1 of these 8 drugs, they may swap between PAH agents at any time without having to re-qualify for treatment with the alternate agent. This means that patients may commence treatment with the alternate agent, subject to that agent's restriction, irrespective of the severity of their disease at the time the application to swap therapy is submitted.
Applications to swap between the 8 PAH agents must be made under the relevant initial treatment (monotherapy) restriction.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Authority Required procedures
C13492 Pulmonary arterial hypertension (PAH)
Transitioning from non-PBS to PBS-subsidised supply of combination therapy (dual or triple therapy, excluding selexipag) - Grandfather arrangements where each drug has not been a PBS benefit
Patient must have been receiving, prior to 1 December 2022, non-PBS-subsidised dual therapy consisting of one endothelin receptor antagonist with one prostanoid, where each drug was not a PBS benefit; this authority application is to continue such combination therapy; OR
Patient must have been receiving, prior to 1 December 2022, non-PBS-subsidised triple therapy consisting of one endothelin receptor antagonist, one prostanoid, one phosphodiesterase-5 inhibitor, where each drug was not a PBS benefit; this authority application is to continue such combination therapy; AND
The condition must have, prior to the time non-PBS combination therapy was initiated, progressed to at least Class III PAH despite treatment with at least one drug from the drug classes mentioned above; OR
The condition must have, at the time non-PBS combination therapy was initiated, been both: (i) classed as at least Class III PAH, (ii) untreated with any drug from the drug classes mentioned above.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted. 		Compliance with Authority Required procedures
C13505 Pulmonary arterial hypertension (PAH)
Initial 3 - changing to this drug in combination therapy (dual or triple therapy)
The treatment must form part of dual combination therapy consisting of: (i) one prostanoid, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one prostanoid; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid.
Patient must be undergoing existing PBS-subsidised combination therapy with at least this drug in the combination changing; combination therapy is not to commence through this Treatment phase listing; AND
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH. 		Compliance with Authority Required procedures
C13506 Pulmonary arterial hypertension (PAH)
Continuing treatment of combination therapy (dual or triple therapy, excluding selexipag)
The treatment must form part of dual combination therapy consisting of: (i) one prostanoid, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one prostanoid; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid.
Patient must be undergoing continuing treatment of existing PBS-subsidised combination therapy (dual/triple therapy, excluding selexipag), where this drug in the combination remains unchanged from the previous authority application; AND
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH. 		Compliance with Authority Required procedures
C13510 Pulmonary arterial hypertension (PAH)
Initial 1 - starting combination therapy (dual or triple therapy, excluding selexipag) in an untreated patient
Patient must not have received prior PBS-subsidised treatment with a pulmonary arterial hypertension (PAH) agent; AND
Patient must currently have WHO Functional Class III PAH or WHO Functional Class IV PAH; AND
The treatment must form part of dual combination therapy consisting of: (i) one prostanoid, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one prostanoid; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid; triple combination therapy is treating a patient with class IV PAH.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that the test results are of a recency that the PAH physician making this authority application is satisfied that the diagnosis of PAH is current.
(5) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The test results must not be more than 6 months old at the time of application. 		Compliance with Written Authority Required procedures
C13512 Pulmonary arterial hypertension (PAH)
Initial 1 (new patients)
Patient must not have received prior PBS-subsidised treatment with a pulmonary arterial hypertension (PAH) agent; AND
Patient must have WHO Functional Class IV PAH; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that the test results are of a recency that the PAH physician making this authority application is satisfied that the diagnosis of PAH is current.
(5) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The test results must not be more than 6 months old at the time of application.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the Therapeutic Goods Administration (TGA) approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Written Authority Required procedures
C13577 Pulmonary arterial hypertension (PAH)
Continuing treatment
Patient must have received their most recent course of PBS-subsidised treatment with this PAH agent for this condition; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
PAH agents are not PBS-subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Authority Required procedures
C13634 Pulmonary arterial hypertension (PAH)
Initial 2 - starting combination therapy (dual or triple therapy, excluding selexipag) in a treated patient where a diagnosis of pulmonary arterial hypertension is established through a prior PBS authority application
Patient must currently have WHO Functional Class III PAH or WHO Functional Class IV PAH; AND
Patient must have failed to achieve/maintain WHO Functional Class II status with at least one of the following PBS-subsidised therapies: (i) endothelin receptor antagonist monotherapy, (ii) phosphodiesterase-5 inhibitor monotherapy, (iii) prostanoid monotherapy; AND
The treatment must form part of dual combination therapy consisting of: (i) one prostanoid, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one prostanoid; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid; triple combination therapy is treating a patient in whom monotherapy/dual combination therapy has been inadequate.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH. 		Compliance with Authority Required procedures

  1. Schedule 3, entry for Riociguat

(a)omit:

C10231 Pulmonary arterial hypertension (PAH)
Continuing treatment
Patient must have received their most recent course of PBS‑subsidised treatment with this PAH agent for this condition; AND
The treatment must be the sole PBS‑subsidised PAH agent for this condition.
The term ‘PAH agents’ refers to bosentan monohydrate, iloprost trometamol, epoprostenol sodium, sildenafil citrate, ambrisentan, tadalafil, macitentan, and riociguat.
PAH agents are not PBS‑subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA‑approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Authority Required procedures
C10243 Pulmonary arterial hypertension (PAH)
Initial 2 (change)
Patient must have documented WHO Functional Class III PAH or WHO Functional Class IV PAH; AND
Patient must have had their most recent course of PBS‑subsidised treatment for this condition with a PAH agent other than this agent; AND
The treatment must be the sole PBS‑subsidised PAH agent for this condition.
The term ‘PAH agents’ refers to bosentan monohydrate, iloprost trometamol, epoprostenol sodium, sildenafil citrate, ambrisentan, tadalafil, macitentan, and riociguat.
PAH agents are not PBS‑subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
Swapping between PAH agents: Patients can access PAH agents through the PBS according to the relevant restrictions. Once these patients are approved initial treatment (monotherapy) with 1 of these 8 drugs, they may swap between PAH agents at any time without having to re‑qualify for treatment with the alternate agent. This means that patients may commence treatment with the alternate agent, subject to that agent’s restriction, irrespective of the severity of their disease at the time the application to swap therapy is submitted.
Applications to swap between the 8 PAH agents must be made under the relevant initial treatment (monotherapy) restriction.
Approvals for prescriptions for dose titration will provide sufficient quantity for dose titrations by 0.5 mg increments at 2‑week intervals to achieve up to a maximum of 2.5 mg three times daily based on the dosage recommendations for initiation of treatment in the TGA‑approved Product Information. No repeats will be authorised for these prescriptions.
Approvals for subsequent authority prescription will be limited to 1 month of treatment, with the quantity approved based on the dosage recommendations in the TGA‑approved Product Information, and a maximum of 4 repeats. 		Compliance with Authority Required procedures
C10245 Pulmonary arterial hypertension (PAH)
Initial 1 (new patients)
Patient must not have received prior PBS‑subsidised treatment with a pulmonary arterial hypertension (PAH) agent; AND
Patient must have been assessed by a physician with expertise in the management of PAH; AND
Patient must have WHO Functional Class III PAH or WHO Functional Class IV PAH; AND
The treatment must be the sole PBS‑subsidised PAH agent for this condition.
The term ‘PAH agents’ refers to bosentan monohydrate, iloprost trometamol, epoprostenol sodium, sildenafil citrate, ambrisentan, tadalafil, macitentan, and riociguat.
PAH agents are not PBS‑subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
PAH (WHO Group 1 pulmonary hypertension) is defined as follows:
(i) mean pulmonary artery pressure (mPAP) greater than or equal to 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) less than or equal to 15 mmHg; or
(ii) where a right heart catheter (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure (RVSP), assessed by echocardiography (ECHO), greater than 40 mmHg, with normal left ventricular function.
Applications for authorisation must be in writing and must include:
(1) a completed authority prescription form; and
(2) a completed Pulmonary Arterial Hypertension PBS Authority Application ‑ Supporting Information form which includes results from the three tests below, where available:
(i) RHC composite assessment; and
(ii) ECHO composite assessment; and
(iii) 6 Minute Walk Test (6MWT).
Where it is not possible to perform all 3 tests on clinical grounds, the following list outlines the preferred test combination, in descending order, for the purposes of initiation of PBS‑subsidised treatment:
(1) RHC plus ECHO composite assessments;
(2) RHC composite assessment plus 6MWT;
(3) RHC composite assessment only.
In circumstances where a RHC cannot be performed on clinical grounds, applications may be submitted for consideration based on the results of the following test combinations, which are listed in descending order of preference:
(1) ECHO composite assessment plus 6MWT;
(2) ECHO composite assessment only.
Where fewer than 3 tests are able to be performed on clinical grounds, a patient specific reason outlining why the particular test(s) could not be conducted must be provided with the authority application.
Where a RHC cannot be performed on clinical grounds, confirmation of the reason(s) must be provided with the authority application by a second PAH physician or cardiologist with expertise in the management of PAH.
The test results provided must not be more than 2 months old at the time of application.
Approvals for prescriptions for dose titration will provide sufficient quantity for dose titrations by 0.5 mg increments at 2‑week intervals to achieve up to a maximum of 2.5 mg three times daily based on the dosage recommendations for initiation of treatment in the TGA‑approved Product Information. No repeats will be authorised for these prescriptions.
Approvals for subsequent authority prescription will be limited to 1 month of treatment, with the quantity approved based on the dosage recommendations in the TGA‑approved Product Information, and a maximum of 4 repeats. 		Compliance with Written Authority Required procedures

(b)insert in numerical order after existing text:

C13502 Pulmonary arterial hypertension (PAH)
Continuing treatment
Patient must have received their most recent course of PBS-subsidised treatment with this PAH agent for this condition; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
PAH agents are not PBS-subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Authority Required procedures
C13514 Pulmonary arterial hypertension (PAH)
Initial 2 (change)
Patient must have documented WHO Functional Class III PAH or WHO Functional Class IV PAH; AND
Patient must have had their most recent course of PBS-subsidised treatment for this condition with a PAH agent other than this agent; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
PAH agents are not PBS-subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
Swapping between PAH agents: Patients can access PAH agents through the PBS according to the relevant restrictions. Once these patients are approved initial treatment (monotherapy) with 1 of these 8 drugs, they may swap between PAH agents at any time without having to re-qualify for treatment with the alternate agent. This means that patients may commence treatment with the alternate agent, subject to that agent's restriction, irrespective of the severity of their disease at the time the application to swap therapy is submitted.
Applications to swap between the 8 PAH agents must be made under the relevant initial treatment (monotherapy) restriction.
Approvals for prescriptions for dose titration will provide sufficient quantity for dose titrations by 0.5 mg increments at 2-week intervals to achieve up to a maximum of 2.5 mg three times daily based on the dosage recommendations for initiation of treatment in the TGA-approved Product Information. No repeats will be authorised for these prescriptions.
Approvals for subsequent authority prescription will be limited to 1 month of treatment, with the quantity approved based on the dosage recommendations in the TGA-approved Product Information, and a maximum of 4 repeats. 		Compliance with Authority Required procedures
C13515 Pulmonary arterial hypertension (PAH)
Initial 1 (new patients)
Patient must not have received prior PBS-subsidised treatment with a pulmonary arterial hypertension (PAH) agent; AND
Patient must have been assessed by a physician with expertise in the management of PAH; AND
Patient must have WHO Functional Class III PAH or WHO Functional Class IV PAH; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that the test results are of a recency that the PAH physician making this authority application is satisfied that the diagnosis of PAH is current.
(5) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The test results must not be more than 6 months old at the time of application.
Approvals for prescriptions for dose titration will provide sufficient quantity for dose titrations by 0.5 mg increments at 2-week intervals to achieve up to a maximum of 2.5 mg three times daily based on the dosage recommendations for initiation of treatment in the TGA-approved Product Information. No repeats will be authorised for these prescriptions.
Approvals for subsequent authority prescription will be limited to 1 month of treatment, with the quantity approved based on the dosage recommendations in the TGA-approved Product Information, and a maximum of 4 repeats. 		Compliance with Written Authority Required procedures

  1. Schedule 3, entry for Sildenafil

substitute:

Sildenafil C11229 Pulmonary arterial hypertension (PAH)
Triple therapy - Initial treatment or continuing treatment of triple combination therapy (including dual therapy in lieu of triple therapy) that includes selexipag
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) PBS-subsidised selexipag (referred to as 'triple therapy'); OR
The treatment must form part of dual combination therapy consisting of either: (i) PBS-subsidised selexipag with one endothelin receptor antagonist, (ii) PBS-subsidised selexipag with one phosphodiesterase-5 inhibitor, as triple combination therapy with selexipag-an endothelin receptor antagonist-a phoshodiesterase-5 inhibitor is not possible due to an intolerance/contraindication to the endothelin receptor antagonist class/phosphodiesterase-5 inhibitor class (referred to as 'dual therapy in lieu of triple therapy').
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
The authority application for selexipag must be approved prior to the authority application for this agent.
For the purposes of PBS subsidy, an endothelin receptor antagonist is one of: (a) ambrisentan, (b) bosentan, (c) macitentan; a phosphodiesterase-5 inhibitor is one of: (d) sildenafil, (e) tadalafil.
PBS-subsidy does not cover patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
PAH (WHO Group 1 pulmonary hypertension) is defined as follows:
(i) mean pulmonary artery pressure (mPAP) greater than or equal to 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) less than or equal to 15 mmHg; or
(ii) where a right heart catheter (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure (RVSP), assessed by echocardiography (ECHO), greater than 40 mmHg, with normal left ventricular function.
The results and date of the RHC, ECHO and 6 MWT as applicable must be included in the patient's medical record. Where a RHC cannot be performed on clinical grounds, the written confirmation of the reasons why must also be included in the patient's medical record.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Authority Required procedures
C13482 Pulmonary arterial hypertension (PAH)
Initial 2 (change)
Patient must have documented WHO Functional Class II PAH, or WHO Functional Class III PAH; AND
Patient must have had their most recent course of PBS-subsidised treatment for this condition with a PAH agent other than this agent; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
PAH agents are not PBS-subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
Swapping between PAH agents: Patients can access PAH agents through the PBS according to the relevant restrictions. Once these patients are approved initial treatment (monotherapy) with 1 of these 8 drugs, they may swap between PAH agents at any time without having to re-qualify for treatment with the alternate agent. This means that patients may commence treatment with the alternate agent, subject to that agent's restriction, irrespective of the severity of their disease at the time the application to swap therapy is submitted.
Applications to swap between the 8 PAH agents must be made under the relevant initial treatment (monotherapy) restriction.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Authority Required procedures
C13484 Pulmonary arterial hypertension (PAH)
Initial 1 (new patients)
Patient must not have received prior PBS-subsidised treatment with a pulmonary arterial hypertension (PAH) agent.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
Patient must have WHO Functional Class II PAH, or WHO Functional Class III PAH; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that the test results are of a recency that the PAH physician making this authority application is satisfied that the diagnosis of PAH is current.
(5) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The test results must not be more than 6 months old at the time of application.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Written Authority Required procedures
C13569 Pulmonary arterial hypertension (PAH)
Initial 3 - changing to this drug in combination therapy (dual or triple therapy)
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one prostanoid, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid.
Patient must be undergoing existing PBS-subsidised combination therapy with at least this drug in the combination changing; combination therapy is not to commence through this Treatment phase listing; AND
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH. 		Compliance with Authority Required procedures
C13570 Pulmonary arterial hypertension (PAH)
Continuing treatment of combination therapy (dual or triple therapy, excluding selexipag)
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one prostanoid, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid.
Patient must be undergoing continuing treatment of existing PBS-subsidised combination therapy (dual/triple therapy, excluding selexipag), where this drug in the combination remains unchanged from the previous authority application; AND
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH. 		Compliance with Authority Required procedures
C13572 Pulmonary arterial hypertension (PAH)
Continuing treatment
Patient must have received their most recent course of PBS-subsidised treatment with this PAH agent for this condition; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
PAH agents are not PBS-subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Authority Required procedures
C13573 Pulmonary arterial hypertension (PAH)
Initial 2 - starting combination therapy (dual or triple therapy, excluding selexipag) in a treated patient where a diagnosis of pulmonary arterial hypertension is established through a prior PBS authority application
Patient must currently have WHO Functional Class III PAH or WHO Functional Class IV PAH; AND
Patient must have failed to achieve/maintain WHO Functional Class II status with at least one of the following PBS-subsidised therapies: (i) endothelin receptor antagonist monotherapy, (ii) phosphodiesterase-5 inhibitor monotherapy, (iii) prostanoid monotherapy; AND
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one prostanoid, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid; triple combination therapy is treating a patient in whom monotherapy/dual combination therapy has been inadequate.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH. 		Compliance with Authority Required procedures
C13629 Pulmonary arterial hypertension (PAH)
Initial 1 - combination therapy (dual or triple therapy, excluding selexipag) in an untreated patient
Patient must not have received prior PBS-subsidised treatment with a pulmonary arterial hypertension (PAH) agent; AND
Patient must currently have WHO Functional Class III PAH or WHO Functional Class IV PAH; AND
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one prostanoid, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid; triple combination therapy is treating a patient with class IV PAH.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted. 		Compliance with Authority Required procedures
C13671 Pulmonary arterial hypertension (PAH)
Transitioning from non-PBS to PBS-subsidised supply of combination therapy (dual or triple therapy, excluding selexipag) - Grandfather arrangements where each drug has not been a PBS benefit
Patient must have been receiving, prior to 1 December 2022, non-PBS-subsidised dual therapy consisting of one endothelin receptor antagonist with one phosphodiesterase-5 inhibitor, where each drug was not a PBS benefit; this authority application is to continue such combination therapy; OR
Patient must have been receiving, prior to 1 December 2022, non-PBS-subsidised triple therapy consisting of one endothelin receptor antagonist, one prostanoid, one phosphodiesterase-5 inhibitor, where each drug was not a PBS benefit; this authority application is to continue such combination therapy; AND
The condition must have, prior to the time non-PBS combination therapy was initiated, progressed to at least Class III PAH despite treatment with at least one drug from the drug classes mentioned above; OR
The condition must have, at the time non-PBS combination therapy was initiated, been both: (i) classed as at least Class III PAH, (ii) untreated with any drug from the drug classes mentioned above.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted. 		Compliance with Authority Required procedures

  1. Schedule 3, entry for Tadalafil

substitute:

Tadalafil C11229 Pulmonary arterial hypertension (PAH)
Triple therapy - Initial treatment or continuing treatment of triple combination therapy (including dual therapy in lieu of triple therapy) that includes selexipag
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) PBS-subsidised selexipag (referred to as 'triple therapy'); OR
The treatment must form part of dual combination therapy consisting of either: (i) PBS-subsidised selexipag with one endothelin receptor antagonist, (ii) PBS-subsidised selexipag with one phosphodiesterase-5 inhibitor, as triple combination therapy with selexipag-an endothelin receptor antagonist-a phoshodiesterase-5 inhibitor is not possible due to an intolerance/contraindication to the endothelin receptor antagonist class/phosphodiesterase-5 inhibitor class (referred to as 'dual therapy in lieu of triple therapy').
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
The authority application for selexipag must be approved prior to the authority application for this agent.
For the purposes of PBS subsidy, an endothelin receptor antagonist is one of: (a) ambrisentan, (b) bosentan, (c) macitentan; a phosphodiesterase-5 inhibitor is one of: (d) sildenafil, (e) tadalafil.
PBS-subsidy does not cover patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
PAH (WHO Group 1 pulmonary hypertension) is defined as follows:
(i) mean pulmonary artery pressure (mPAP) greater than or equal to 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) less than or equal to 15 mmHg; or
(ii) where a right heart catheter (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure (RVSP), assessed by echocardiography (ECHO), greater than 40 mmHg, with normal left ventricular function.
The results and date of the RHC, ECHO and 6 MWT as applicable must be included in the patient's medical record. Where a RHC cannot be performed on clinical grounds, the written confirmation of the reasons why must also be included in the patient's medical record.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Authority Required procedures
C13482 Pulmonary arterial hypertension (PAH)
Initial 2 (change)
Patient must have documented WHO Functional Class II PAH, or WHO Functional Class III PAH; AND
Patient must have had their most recent course of PBS-subsidised treatment for this condition with a PAH agent other than this agent; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
PAH agents are not PBS-subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
Swapping between PAH agents: Patients can access PAH agents through the PBS according to the relevant restrictions. Once these patients are approved initial treatment (monotherapy) with 1 of these 8 drugs, they may swap between PAH agents at any time without having to re-qualify for treatment with the alternate agent. This means that patients may commence treatment with the alternate agent, subject to that agent's restriction, irrespective of the severity of their disease at the time the application to swap therapy is submitted.
Applications to swap between the 8 PAH agents must be made under the relevant initial treatment (monotherapy) restriction.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Authority Required procedures
C13484 Pulmonary arterial hypertension (PAH)
Initial 1 (new patients)
Patient must not have received prior PBS-subsidised treatment with a pulmonary arterial hypertension (PAH) agent.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
Patient must have WHO Functional Class II PAH, or WHO Functional Class III PAH; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that the test results are of a recency that the PAH physician making this authority application is satisfied that the diagnosis of PAH is current.
(5) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The test results must not be more than 6 months old at the time of application.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Written Authority Required procedures
C13569 Pulmonary arterial hypertension (PAH)
Initial 3 - changing to this drug in combination therapy (dual or triple therapy)
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one prostanoid, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid.
Patient must be undergoing existing PBS-subsidised combination therapy with at least this drug in the combination changing; combination therapy is not to commence through this Treatment phase listing; AND
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH. 		Compliance with Authority Required procedures
C13570 Pulmonary arterial hypertension (PAH)
Continuing treatment of combination therapy (dual or triple therapy, excluding selexipag)
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one prostanoid, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid.
Patient must be undergoing continuing treatment of existing PBS-subsidised combination therapy (dual/triple therapy, excluding selexipag), where this drug in the combination remains unchanged from the previous authority application; AND
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH. 		Compliance with Authority Required procedures
C13572 Pulmonary arterial hypertension (PAH)
Continuing treatment
Patient must have received their most recent course of PBS-subsidised treatment with this PAH agent for this condition; AND
The treatment must be the sole PBS-subsidised PAH agent for this condition.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.
PAH agents are not PBS-subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted.
The maximum quantity authorised will be limited to provide sufficient supply for 1 month of treatment, based on the dosage recommendations in the TGA-approved Product Information.
A maximum of 5 repeats may be requested. 		Compliance with Authority Required procedures
C13573 Pulmonary arterial hypertension (PAH)
Initial 2 - starting combination therapy (dual or triple therapy, excluding selexipag) in a treated patient where a diagnosis of pulmonary arterial hypertension is established through a prior PBS authority application
Patient must currently have WHO Functional Class III PAH or WHO Functional Class IV PAH; AND
Patient must have failed to achieve/maintain WHO Functional Class II status with at least one of the following PBS-subsidised therapies: (i) endothelin receptor antagonist monotherapy, (ii) phosphodiesterase-5 inhibitor monotherapy, (iii) prostanoid monotherapy; AND
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one prostanoid, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid; triple combination therapy is treating a patient in whom monotherapy/dual combination therapy has been inadequate.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH. 		Compliance with Authority Required procedures
C13629 Pulmonary arterial hypertension (PAH)
Initial 1 - combination therapy (dual or triple therapy, excluding selexipag) in an untreated patient
Patient must not have received prior PBS-subsidised treatment with a pulmonary arterial hypertension (PAH) agent; AND
Patient must currently have WHO Functional Class III PAH or WHO Functional Class IV PAH; AND
The treatment must form part of dual combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of dual combination therapy consisting of: (i) one prostanoid, (ii) one phosphodiesterase-5 inhibitor; OR
The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) one prostanoid; triple combination therapy is treating a patient with class IV PAH.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted. 		Compliance with Authority Required procedures
C13671 Pulmonary arterial hypertension (PAH)
Transitioning from non-PBS to PBS-subsidised supply of combination therapy (dual or triple therapy, excluding selexipag) - Grandfather arrangements where each drug has not been a PBS benefit
Patient must have been receiving, prior to 1 December 2022, non-PBS-subsidised dual therapy consisting of one endothelin receptor antagonist with one phosphodiesterase-5 inhibitor, where each drug was not a PBS benefit; this authority application is to continue such combination therapy; OR
Patient must have been receiving, prior to 1 December 2022, non-PBS-subsidised triple therapy consisting of one endothelin receptor antagonist, one prostanoid, one phosphodiesterase-5 inhibitor, where each drug was not a PBS benefit; this authority application is to continue such combination therapy; AND
The condition must have, prior to the time non-PBS combination therapy was initiated, progressed to at least Class III PAH despite treatment with at least one drug from the drug classes mentioned above; OR
The condition must have, at the time non-PBS combination therapy was initiated, been both: (i) classed as at least Class III PAH, (ii) untreated with any drug from the drug classes mentioned above.
Must be treated by a physician with expertise in the management of PAH, with this authority application to be completed by the physician with expertise in PAH.
Applications for authorisation of initial treatment must be made via the Online PBS Authorities System (real time assessment) or in writing via HPOS form upload or mail.
If the application is submitted through HPOS form upload or mail, it must include:
(a) a completed authority prescription form; and
(b) a completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice).
(1) Confirm that the patient has a diagnosis of pulmonary arterial hypertension (PAH) in line with the following definition:
(a) mean pulmonary artery pressure (mPAP) at least 25 mmHg at rest and pulmonary artery wedge pressure (PAWP) no greater than 15 mmHg; or
(b) where right heart catheterisation (RHC) cannot be performed on clinical grounds, right ventricular systolic pressure assessed by echocardiography (ECHO) is greater than 40 mmHg, with normal left ventricular function.
(2) Confirm that in forming the diagnosis of PAH, the following tests have been conducted:
- RHC composite assessment; and
- ECHO composite assessment; and
- 6 Minute Walk Test (6MWT)
Where it is not possible to perform all 3 tests on clinical grounds, the expected test combination, in descending order, is:
- RHC plus ECHO composite assessments;
- RHC composite assessment plus 6MWT;
- RHC composite assessment only.
In circumstances where RHC cannot be performed on clinical grounds, the expected test combination, in descending order, is:
- ECHO composite assessment plus 6MWT;
- ECHO composite assessment only.
(3) Document the findings of these tests in the patient's medical records, including, where relevant only, the reason/s:
(i) for why fewer than 3 tests are able to be performed on clinical grounds;
(ii) why RHC cannot be performed on clinical grounds - confirm this by obtaining a second opinion from another PAH physician or cardiologist with expertise in the management of PAH; document that this has occurred in the patient's medical records.
(4) Confirm that this authority application is not seeking subsidy for a patient with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of predicted. 		Compliance with Authority Required procedures
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