A patient’s first treatment must be monotherapy. A patient is expected to start on sildenafil
monotherapy, unless they are:
a) A child with idiopathic PAH or PAH secondary to congenital heart disease, in which
case they may start with bosentan monotherapy.
b) Intolerant of or contraindicated to sildenafil, in which case they may have bosentan
monotherapy, or may apply to the Panel for ambrisentan, iloprost, or epoprostenol
monotherapy. (Epoprostenol monotherapy may only be applied for in patients meeting
the criteria described further below.)
A patient will need to complete three months of a monotherapy treatment before combination
treatments will be considered.
To be eligible for dual therapy, a patient must either have:
• Tried a monotherapy for three months with no response, or
• Have deteriorated while on a monotherapy, as determined by:
o Clear evidence of deterioration in right heart cardiac catheterisation measures;
o 15% deterioration in two 6 minute walk tests (6MWTs) done at least two weeks
o NYHA/WHO functional class IV (the NYHA/WHO functional classification for
PAH replicated on page 4).
For patients who are tolerant of sildenafil, one of the two agents must be sildenafil. Use of
epoprostenol is restricted as described further below. Beyond that, clinicians may apply for any
If a patient does not respond to a particular dual combination or deteriorates as described
above, the clinician may apply for another dual therapy, noting the above restrictions still apply.
Triple therapy for patients on the lung transplant list
A patient on the lung transplant waiting list may apply for triple therapy. Any combination may
be applied for, as long as sildenafil-tolerant patients include sildenafil as one of the three
Triple therapy for patients for not on lung transplant list
A patient not on the lung transplant list may apply for triple therapy if:
a) The triple therapy would consist of sildenafil, bosentan, and any one other agent, and;
b) The patient meets any one of the following three criteria:
1. Patient presents acutely with idiopathic pulmonary arterial hypertension (IPAH) in New
York Heart Association/World Health Organization (NYHA/WHO) Functional Class IV; or
2. Patient is deteriorating rapidly to NYHA/WHO Functional Class IV and may be a lung
transplant recipient in the future, if their disease is stabilised; or
3. Patient has PAH associated with the scleroderma spectrum of diseases (APAHSSD)
who have no major morbidities and are deteriorating despite combination therapy.
QUARDUPLE AND QUINTUPLE THERAPY
Treatments with four or more PAH agents are not funded.