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

Pulmonary arterial hypertension (PAH)

Initial treatment 1 - New patient

Patient must not have received prior PBS-subsidised treatment with a pulmonary arterial hypertension (PAH) agent; AND

The treatment must be the sole PBS-subsidised PAH agent for this condition; 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.

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) 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.

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.

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

Pulmonary arterial hypertension (PAH)

Initial treatment 2 - Change of treatment

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; 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.

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.

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

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; 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.

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.