SA0967 - Endothelian receptor antagonists (text extract)

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ELIGIBILITY CRITERIA FOR PULMONARY ARTERIAL HYPERTENSION THERAPY

Contents

ELIGIBILITY CRITERIA FOR INITIATION OF PULMONARY ARTERIAL HYPERTENSION THERAPY ............................................................................................................................. 2 Initial Application for Funding of Pulmonary Arterial Hypertension Treatments for Adults (and children aged over 10 years) ................................................................................................. 6 Renewal Application / Request for Change of Therapy for Pulmonary Arterial Hypertension Treatments for Adults (and children aged over 10 years) .................................................... 13 Initial Application for Funding of Pulmonary Arterial Hypertension Treatment Application for children less than 10 years for PDE-5 inhibitor (sildenafil) treatment ................................... 17 Renewal Application for Funding of Pulmonary Arterial Hypertension Treatment . Application for children less than 10 years for PDE-5 inhibitor (sildenafil) treatment .............................. 22

ELIGIBILITY CRITERIA FOR INITIATION OF PULMONARY ARTERIAL HYPERTENSION THERAPY

These guidelines are intended to assist relevant practitioners in gauging which patients are likely to be approved for pulmonary arterial hypertension treatments. In view of the complexity of pulmonary arterial hypertension diagnosis, classification and severity assessment, each application is thoroughly evaluated by the PAH Panel to determine the appropriateness of pulmonary vasodilator treatment. Please note that the pharmaceuticals covered by these access criteria have different registered indications, and that some of the criteria included here are Unapproved Indications. If clinicians are intending to prescribe any of these pharmaceuticals for an Unapproved Indication, they should be aware of and comply with their obligations, including those set out in rule 4.6 of the Pharmaceutical Schedule. All requested studies should be carried out in line with the relevant professional guidelines. Patients with pulmonary arterial hypertension who meet the following criteria may be eligible for initiation of pulmonary arterial hypertension treatment based on current clinical evidence. The following treatments may be subsidised by application to the PAH Panel:

Category Endothelin receptor antagonists Phosphodiesterase type 5 inhibitors Prostacyclin analogues Treatment Ambrisentan (Volibris) Bosentan (Tracleer) Sildenafil (Viagra) Iloprost (Ventavis)

Patients eligible for initial approval of Special Authority

1. The patient must have a diagnosis of pulmonary arterial hypertension with the following WHO (Venice) clinical classifications: · Group 1 § Idiopathic; § Familial; § Associated with: Connective tissue disease; Congenital systemic pulmonary shunts; Portal hypertension; HIV infection; Drugs and toxins; Other; § Associated with significant venous or capillary involvement: Pulmonary veno-occlusive disease (PVOD); Pulmonary capillary haemangiomatosis (PCH); § Persistent pulmonary hypertension of the newborn (PPHN) · Group 4 § Pulmonary arterial hypertension due to thrombotic and/or embolic disease only) · Group 5 § Miscellaneous group E.g. sarcoidosis, histiocytosis X and lymphangiomatosis Patients with PAH classified as group 2 or 3 are not eligible for subsidised treatment. · Group 2 - pulmonary hypertension associated with left heart disease · Group 3 - pulmonary hypertension associated with respiratory diseases and / or hypoxaemia Lung function tests and cardiac function tests must be supplied with the initial application. For children under 10 years old, funding of sildenafil monotherapy will be considered using the data provided according to the application forms for children less than 10 years. 2. The patient must have NYHA/WHO functional class III or IV. For patients who are functional class II, applications will be considered in cases where there is clear evidence of disease progression (defined as a deterioration in performance of the 6MWT or deterioration in haemodynamic variables) despite current therapy. Patients who are functional class I are not eligible for subsidised treatment.

New York Heart Association / World Health Organization Functional Classification of Pulmonary Hypertension Class I: Class II: Patients with pulmonary hypertension but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnoea or fatigue, chest pain, or near syncope. Patients with pulmonary hypertension resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity does not cause undue

dyspnoea or fatigue, chest pain, or near syncope. Class III: Patients with pulmonary hypertension resulting in pronounced limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnoea or fatigue, chest pain, or near syncope. Patients with pulmonary hypertension with inability to carry out any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnoea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity.

Class IV:

3. Right cardiac catheterisation data1 must be supplied with the application. If cardiac catheterisation is contra-indicated, a letter of explanation is required. Unequivocal, significant evidence of raised pulmonary arterial pressure, in the absence of significant left heart disease, must be demonstrated. · The patient must have a pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg (patients with a PCWP between 15 mmHg and 18 mmHg may be considered at the Panel’s discretion). · The patient must have a mean pulmonary artery pressure (PAPm) > 25 mmHg. · An assessment of vasoreactivity has been carried out using iloprost, adenosine or nitric oxide. Where this assessment has not been carried out, applicants must provide reasons for this. (Vasoreactivity studies are not mandatory in patients with severe PAH (functional class IV or right atrial pressure > 12 mmHg or Cardiac Index < 2 l/min/m2) or PAH associated with connective tissue disease.) · Where the patient has been shown to be vasoreactive (defined as a fall in mean PAP of greater than or equal to 10mmHg to less than 40mmHg with either an increase or no change in cardiac index), evidence of an adequate therapeutic trial of calcium channel blockers for three to six months must have been undertaken, followed by re-catheterisation demonstrating evidence of haemodynamic progression. (Due to the negative inotropic effects of CCBs, a trial of CCBs is not required in patients with severe disease as defined above.) Treatment selection Initial treatment: § Patients who have not previously been treated with any of these agents would generally be expected to start treatment with sildenafil. NYHA WHO functional class IV patients who have been stabilised in hospital on iloprost would be able to receive iloprost in the community for a period of time to allow transition to sildenafil monotherapy, if clinically appropriate.

Change of treatment: § Where sildenafil is not tolerated due to side-effects clinicians may apply for monotherapy with bosentan, ambrisentan or iloprost.

Grossman, W (Ed). Cardiac Catheterization and Angiography, 3rd ed, Lea & Febiger, Philadelphia 1986

Where the patient has not responded to sildenafil monotherapy, clinicians may apply for alternative monotherapy within 6 months of treatment initiation.

Patients eligible for renewal of Special Authority

Renewal applications must be submitted to the PAH Panel after six months of treatment approval, with annual reviews required thereafter. Treatment selection Renewal of treatment (stable patients): § Patients who are stable or improve on PAH therapy will be considered for renewal of special authority.

Escalation of treatment: § Eligibility for combination therapy for patients stable on treatment for at least six months who then deteriorate shall be determined by: · Clear evidence of deterioration in right heart cardiac catheterisation measures; or · 15% deterioration in two 6MWTs done at least two weeks apart; or · NYHA/WHO functional class IV. § Where patients show signs and symptoms of deterioration, and escalation of treatment is requested, a repeat right heart cardiac catheter is mandatory, except if the cardiac catheter is contraindicated, when a letter of explanation must be provided for the Panel to consider. Patients who have failed to respond to two monotherapies within the first six months of treatment may be eligible for combination therapy. Combination bosentan/iloprost or ambrisentan/iloprost may be considered for patients who cannot tolerate a sildenafil regime. Combination sildenafil/bosentan/iloprost therapy will not be approved. Combination sildenafil/ambrisentan/iloprost therapy will not be approved.

§ § § §

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

Date of Application __________________

Please send applications to:

PAH Panel Coordinator PHARMAC P O Box 10-254 WELLINGTON Phone: 04 9167 512 Facsimile: 04 974 4858 Email: PAH@pharmac.govt.nz

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

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

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:

Treatment requested q Endothelin receptor antagonists [ambrisentan, 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, ambrisentan 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: __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ __________________________________________________________________________ _______________________________________________________

Basis of request for PAH treatments Diagnosis Patient has been diagnosed as having pulmonary arterial hypertension NYHA/WHO functional class *2 q 3 q 4 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

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

Lung function Date of test:_________________ Please report as actual values and percent predicted and x attach report Actual FEV1 FVC FEV1/FVC (%) DLCO DLCO/VA TLC Six minute walk test: Date of 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

Right Heart Cardiac Catheter (please attach reports) Date of test: 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:

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

Date of test:

Medical History q Not suitable for referral/turned down Transplant status: q Not yet referred q Inactive waiting list q Active waiting list

Comorbidities:

Current and relevant medications (including CCBs):

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

Renewal Application / Request for Change of Therapy for Pulmonary Arterial Hypertension Treatments for Adults (and children aged over 10 years)

Use this form for renewal applications and applications for therapy changes. Initial approval is valid for a period of six months. Subsequent approvals are valid for twelve months. Date of Application:_________________

Please send applications to:

PAH Panel Coordinator PHARMAC P O Box 10-254 WELLINGTON Phone: 04 9167 512 Facsimile: 04 974 4858 Email : PAH@pharmac.govt.nz

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

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

q q

Cardiac catheterisation report Echo results

Phone No: Email:

Home:

Work:

Mobile:

Patient’s Physician Name: NZMC Registration Number: Practice Address:

Phone No: Mobile No: Fax No: Email:

Signature of applying physician:

Date:

Current treatment and dosing: _____________________________________________ Treatment requested q Endothelin receptor antagonists [bosentan, ambrisentan] q Phosphodiesterase type-5 inhibitors [sildenafil] q Prostacyclin analogues [iloprost] Are you applying for a change in therapy? Are you applying for combination treatment? Are you applying for a renewal of current treatment only? q Yes q Yes q Yes q No q No q No

If applying for a change of therapy or combination therapy, please indicate reasons for change: Intolerance of current treatment (please provide details below) Lack of response to current treatment (please provide details below) Disease progression following previous disease stability (please provide details below) Please discuss the rationale for requesting a change in treatment: q q q

Note: · · · · Where the patient has not responded to sildenafil monotherapy, clinicians may apply for alternative monotherapy within 6 months of treatment initiation. Combination sildenafil/bosentan, sildenafil/ambrisentan, or sildenafil/iloprost therapy may be approved after failure of monotherapy Combination bosentan/iloprost therapy or combination ambrisentan/iloprost therapy will not be approved, except for patients with documented intolerance of sildenafil. Triple therapy (bosentan/sildenafil/iloprost or ambrisentan/sildenafil/iloprost) will not be approved.

Status Update NYHA/WHO functional class 2 q 3 q 4 q

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

Six minute walk test (x2 if annual renewal ie done every six months): Distance walked (m): SpO2: Heart Rate: Borg Index: Baseline: Baseline: Pre: Nadir: Maximum: Post:

Brain natriuretic peptide if available – please provide reference data:

Right heart cardiac catheter (please attach report) Date of test:

All patients: Repeat cardiac catheter reports must be provided one year after the start of treatment. § Stable patients: cardiac catheter reports are required at 2 to 4 year intervals depending upon patient progress. § Unstable patients: Where escalation of treatment is requested, a repeat right heart cardiac catheter is mandatory.

Testing centre: Pulmonary capillary wedge pressure: (Threshold: ≤ 15 mmHg) Mean: Pulmonary artery pressures: Systolic: Diastolic: Mean right atrial pressure: Pulmonary vascular resistance: Cardiac output q Wood units q International units

Cardiac index

Cardiac catheter contraindicated: Discussion:

Echocardiography (please attach full report) Echo RVP Echo RAP

Date of test:

Current symptoms / general well-being over previous 6 or 12 months (as applicable)

Initial Application for Funding of Pulmonary Arterial Hypertension Treatment Application for children less than 10 years for PDE-5 inhibitor (sildenafil) treatment

This form is applicable for younger patients requiring PDE-5 inhibitor (sildenafil) monotherapy only. If other treatments are required use the form for adults and children aged over 10 years. Date of Application:_______________

Please send applications to:

PAH Panel Coordinator PHARMAC P O Box 10-254 WELLINGTON Phone: Facsimile: Email: 04 9167 512 04 974 4858 PAH@pharmac.govt.nz

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

q q q

Cardiac catheterisation report Echocardiography report CT report

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: Treatment requested q Phosphodiesterase type-5 inhibitors [sildenafil]

q Suspension required. Please indicate which suspension product will be used: q Methycellulose or water. Available via community funding, dispensed by HP1 community xxxpharmacies and hospital pharmacies. q Ora-Blend or Ora-Plus or Ora-Sweet. Available via DHB Hospital funding, dispensed only by xxxhospital pharmacies. Please ensure that the funding DHB hospital has agreed to pay for this xxxsuspending product.

Please describe anticipated benefits of treatment _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________ _________________________________________________________________________

Basis of request for PAH treatments Diagnosis Patient has been diagnosed as having pulmonary arterial hypertension 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 Tick q

Test results Height (cm): Height centile: Gestation at birth: Number of hospital admissions: ICU Days: History of ventilation: Current oxygen use: Saturations Results of overnight oximetry: Chest X Ray findings: Six minute walk test: (if relevant) Distance walked (m): SpO2 Heart Rate Borg Index Other: Brain natriuretic peptide if available – please provide reference data: Radiology CT Chest results – if applicable (attach report) Baseline: Baseline: Pre: Nadir: Maximum: Post: Date of test: On Room air: _____ On Oxygen at ___ Litres/min: ____ Weight (kg): Weight centile: Birth weight (centile):

Right heart cardiac catheterisation (if performed) Testing centre: Pulmonary capillary wedge pressure: Pulmonary artery pressures: Mean: Systolic: Mean right atrial pressure: Pulmonary vascular resistance – Wood units: Cardiac output: Cardiac index: Cardiac catheter contraindicated: Discussion:

Date of test:

Threshold: ≤ 15 mmHg Threshold > 25 mmHg at rest Diastolic:

q Pulmonary vascular resistance Indexed

Echocardiography (please attach report) Estimate of PA pressure How obtained? (TR jet vs PDA or other) Systemic BP RV dilation RV function Structural congenital heart disease (please describe)

Date of test:

Renewal Application for Funding of Pulmonary Arterial Hypertension Treatment . Application for children less than 10 years for PDE-5 inhibitor (sildenafil) treatment

Please send applications to:

PAH Panel Coordinator PHARMAC P O Box 10-254 WELLINGTON Phone: Facsimile: Email: 04 9167 512 04 974 4858 PAH@pharmac.govt.nz

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

q q q

Echocardiography report Cardiac catheterisation report CT report

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: Treatment requested q Phosphodiesterase type-5 inhibitors [sildenafil]

q Suspension required. Please indicate which suspension product will be used: q Methycellulose or water. Available via community funding, dispensed by HP1 community xxxpharmacies and hospital pharmacies. q Ora-Blend or Ora-Plus or Ora-Sweet. Available via DHB Hospital funding, dispensed only xxxby hospital pharmacies. Please ensure that the funding DHB hospital has agreed to pay for xxxthis suspending product.

Please discuss patient’s progress on treatment and evidence for response: ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________

In addition to anticipated duration, please describe proposed criteria for weaning therapy. Note - If ongoing therapy planned at 1 year of age (or following a year of therapy) it is recommended consideration should be given to risk/benefit of cardiac catheterisation if not performed prior. ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________

Basis of request for PAH treatments Diagnosis Patient has been diagnosed as having pulmonary arterial hypertension 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 Tick q

Test results Height (cm): Height centile: Oxygen Saturations Current oxygen use: Results of overnight oximetry: Hospital admissions since approval (please list dates and diagnoses) ICU days since approval: Chest X Ray findings: Six minute walk test: (if relevant) Distance walked (m): SpO2: Heart Rate: Borg Index: Other: Brain natriuretic peptide if available – please provide reference data: Radiology CT Chest results – if applicable (attach report) Baseline: Baseline: Pre: Nadir: Maximum: Post: Date of test: Weight (kg): Weight centile: On Room air _____ On Oxygen at ___ Litres/min: ____

Right Heart Cardiac Catheter (please attach reports) Date of test: Testing centre:

Date of current catheter: Date of previous catheter:

Pulmonary capillary wedge pressure: (Threshold: ≤ 15 mmHg) Pulmonary artery pressures: (mPAP >25mmHg at rest) Mean right atrial pressure: Pulmonary vascular resistance: Cardiac output Cardiac index Cardiac catheter contraindicated: Discussion: q Wood units Mean: Systolic: Diastolic:

Echocardiography (please attach report)

Date of current echo: Date of previous echo:

Estimate of PA pressure: How obtained? (TR jet vs PDA or other) Systemic BP: RV dilation: RV function: Structural congenital heart disease (please describe):

Metadata

Title

SA0967 - Endothelian receptor antagonists

Abstract

ELIGIBILITY CRITERIA FOR PULMONARY ARTERIAL HYPERTENSION THERAPY Contents ELIGIBILITY CRITERIA FOR INITIATION OF PULMONARY ARTERIAL HYPERTENSION THERAPY ….. 2 Initial Application for Funding of Pulmonary Arterial Hypertension Treatments for Adults (and children aged over 10 years) …. 6 Renewal Application…

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