Listing of Pharmaceutical Benefits (NHL) - Schedule 4 part 1

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

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; this combination therapy is treating a patient with at least WHO functional class III PAH; 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; 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.

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) details of the proposed prescription; 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.

Listing of Pharmaceutical Benefits (NHL) - Schedule 4 part 1

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

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; prior monotherapy has been inadequate - the patient has at least WHO functional class III PAH; 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 - the patient has WHO functional class IV PAH; 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.

Listing of Pharmaceutical Benefits (NHL) - Schedule 4 part 1

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; 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; 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.

Listing of Pharmaceutical Benefits (NHL) - Schedule 4 part 1

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; 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; 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; or

Patient must be undergoing continuing treatment with dual combination therapy consisting of endothelin receptor antagonist with phosphodiesterase-5 inhibitor where one drug was prescribed as a non-PBS benefit.

Listing of Pharmaceutical Benefits (NHL) - Schedule 4 part 1

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'); 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.

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.