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

Pulmonary arterial hypertension (PAH)

Initial treatment following dose titration

Patient must have WHO Functional Class III PAH at treatment initiation with this drug; or

Patient must have WHO Functional Class IV PAH at treatment initiation with this drug; AND

The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) selexipag (referred to as 'triple therapy'); or

The treatment must form part of dual combination therapy consisting of either: (i) selexipag with one endothelin receptor antagonist, (ii) 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

Patient must have completed the dose titration phase; AND

The treatment must not be as monotherapy; 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 have had at least one PBS-subsidised PAH agent prior to this authority application.

Select one appropriate strength (determined under the 'Initial treatment - dose titration' phase) and apply under this treatment phase (Initial treatment following dose titration) once only. Should future dose adjustments be required, apply under the 'Continuing treatment' restriction.

A prior PAH agent is any of: ambrisentan, bosentan, macitentan, sildenafil, tadalafil, epoprostenol, iloprost, riociguat.

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.

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

Pulmonary arterial hypertension (PAH)

Continuing treatment

Patient must have received PBS-subsidised treatment with this drug for this condition; AND

Patient must not have developed disease progression while receiving treatment with this drug for this condition; AND

The treatment must form part of triple combination therapy consisting of: (i) one endothelin receptor antagonist, (ii) one phosphodiesterase-5 inhibitor, (iii) selexipag (referred to as 'triple therapy'); or

The treatment must form part of dual combination therapy consisting of either: (i) selexipag with one endothelin receptor antagonist, (ii) 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

The treatment must not be as monotherapy; 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.

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.

For the purposes of administering this restriction, disease progression has developed if at least one of the following has occurred:

(i) Hospitalisation due to worsening PAH;

(ii) Deterioration of aerobic capacity/endurance, consisting of at least a 15% decrease in 6-Minute Walk Distance from baseline, combined with worsening of WHO functional class status;

(iii) Deterioration of aerobic capacity/endurance, consisting of at least a 15% decrease in 6-Minute Walk Distance from baseline, combined with the need for additional PAH-specific therapy;

(iv) Initiation of parenteral prostanoid therapy or long-term oxygen therapy for worsening of PAH;

(v) Need for lung transplantation or balloon atrial septostomy for worsening of PAH.