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

Pulmonary arterial hypertension (PAH)

Initial treatment - dose titration

Patient must have failed to achieve/maintain a WHO Functional Class II status with PAH agents (other than this agent) given as dual therapy; AND

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 be for dose titration purposes with the intent of completing the titration within 12 weeks; 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; AND

Patient must have had at least one PBS-subsidised PAH agent prior to this authority application.

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.