This is the text extract for 2. Adults Initial PAH application form, browse documents here.

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Initial Application for Funding of Pulmonary Arterial Hypertension Treatments for Adults (and children aged over 10 years)

Please send applications to:

PAH Panel Coordinator PHARMAC P O Box 10-254 WELLINGTON Phone: 04 9167 512 Facsimile: 04 460 4858 Email: PAH@pharmac.govt.nz

Applications must be complete and accompanied by supporting data where required. Have you attached:

q q q q q

Cardiac catheterisation reports Lung function tests Echocardiography report Vasoreactivity data CCB trial results

Patient Details – patient sticker is acceptable Surname: First Name/s: NHI No: Gender: D.O.B: Address: q Male q Female

Phone No: Email:

Home:

Work:

Mobile:

Physician Details Name: NZMC Registration Number: Practice Address:

Phone No: Mobile No: Fax No: Email: Signature of applying physician: Date:

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Treatment requested q Endothelian receptor antagonists [bosentan] q Phosphodiesterase type-5 inhibitors [sildenafil] q Prostacyclin analogues [iloprost] · · · Patients who have not previously been treated with any of these agents would generally be expected to start treatment with sildenafil. Where sildenafil is contraindicated, clinicians may apply for monotherapy with either bosentan or iloprost. If this situation applies, please provide details below. NYHA/WHO functional class IV patients who have been stabilised in hospital on iloprost would be able to receive this in the community for a period of time to allow transition to oral therapy, if clinically appropriate. If this situation applies, please provide details below.

Please discuss the rationale for the proposed treatment regime: _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________

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Basis of request for PAH treatments Diagnosis Patient has been diagnosed as having pulmonary arterial hypertension NYHA/WHO functional class *2 3 4 q q q * Note: Applications for patients with functional class 2 need to demonstrate clear evidence of disease progression on current treatments. Tick q

WHO (Venice) clinical classification Group One – Pulmonary arterial hypertension Idiopathic PAH Familial PAH Associated with other diseases: Connective tissue disease Congenital systemic pulmonary shunts Portal hypertension HIV infection Drugs/toxins Other (specify): Associated with significant venous or capillary involvement Pulmonary veno-occlusive disease Pulmonary capillary haemangiomatosis Persistent pulmonary hypertension of the newborn Group Four – Pulmonary hypertension due to chronic thrombotic and/or embolic disease only Group Five – Other pulmonary hypertension (specify) q q q q q q q q q q q q q

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Test results Height (cm): Weight (kg): BMI (kg/m2):

Lung function (please report as actual values and percent predicted – please attach report): Actual FEV1 FVC FEV1/FVC (%) DLCO DLCO/VA TLC Six minute walk test: Distance walked (m): SpO2: Heart Rate: Borg Index: Brain natriuretic peptide if available – please provide reference data Baseline: Baseline: Pre: Nadir: Maximum: Post: Percent predicted

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Right Heart Cardiac Catheter (please attach reports) Testing centre: Pre vasoreactivity testing Pulmonary capillary wedge pressure: (Threshold: ≤ 15 mmHg): Mean: Pulmonary artery pressures: Systolic: Diastolic: Mean right atrial pressure: Pulmonary vascular resistance: q Wood units q International units Post vasoreactivity testing

Cardiac output: Cardiac index: Vasoreactivity Has the patient been assessed for vasoreactivity using iloprost, adenosine or nitric oxide? If no, please provide reasons: q Yes q No

Cardiac catheterisation contraindicated: Discussion:

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Calcium channel blocker (CCB) history · If the patient has idiopathic PAH and is vasoreactive, and has had a trial of CCBs for at least three months (preferably six), please attach re-catheterisation data demonstrating disease progression despite CCB treatment. · If necessary, please provide discussion of CCB treatment:

__________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________

Echocardiography (please attach full report) Echo RVP Echo RAP

Medical History

q Not suitable for referral/turned down

Transplant status:

q Not yet referred q Inactive waiting list q Active waiting list

Comorbidities:

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Current and relevant medications (including CCBs):

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Would you like to make any other comments in support of this application?