Primary pulmonary and pulmonary arterial hypertension

The Pharmaceutical Benefits Scheme subsidises a range of medicines for patients with primary pulmonary and pulmonary arterial hypertension.

For patients with primary pulmonary hypertension (PPH) and pulmonary arterial hypertension (PAH), treatment with the following PAH agents can be subsidised through the Pharmaceutical Benefits Scheme (PBS) under section 100 of the National Health Act 1953:

  • ambrisentan (Volibris®)
  • bosentan monohydrate (Tracleer®)
  • epoprostenol sodium (Flolan®)
  • iloprost trometamol (Ventavis®)
  • macitentan (Opsumit®)
  • sildenafil citrate (Revatio®), and
  • tadalafil (Adcirca®)

Section 100 arrangements

These items are only available to a patient who is attending either:

  • an approved private hospital
  • a public participating hospital, or
  • a public hospital

and is either a

  • day admitted patient
  • non admitted patient, or
  • patient on discharge

These items are not available as PBS benefits for in-patients of the hospital. The hospital provider number must be included on the application form.

Patient eligibility

Ambrisentan (Volibris®) is available under the PBS as an 'authority required' item for the treatment of:

  • World Health Organization (WHO) functional Class III or IV PPH, and
  • WHO functional Class III or IV PAH secondary to connective tissue disease

Bosentan monohydrate (Tracleer®) is available under the PBS as an 'authority required' item for the treatment of:

  • WHO functional Class III or IV PPH
  • WHO functional Class III or IV PAH secondary to connective tissue disease, and
  • WHO functional Class III or IV PAH associated with a congenital systemic-to-pulmonary shunt, including Eisenmenger's physiology

Epoprostenol sodium (Flolan®) is available under the PBS as an 'authority required' item for the treatment of:

  • WHO functional Class IV PPH
  • WHO functional Class IV PAH secondary to connective tissue disease
  • WHO functional Class III PPH where the patient has previously failed treatment with a PBS-subsidised PAH agent, and
  • WHO functional Class III PAH secondary to connective tissue disease where the patient has previously failed treatment with a PBS-subsidised PAH agent

Iloprost trometamol (Ventavis®) is available under the PBS as an 'authority required' item for the treatment of:

  • WHO functional Class IV PPH
  • WHO functional Class IV PAH secondary to connective tissue disease
  • WHO functional Class III PPH where the patient has previously failed treatment with a PBS-subsidised PAH agent
  • WHO functional Class III PAH secondary to connective tissue disease where the patient has previously failed treatment with a PBS-subsidised PAH agent, and
  • WHO functional Class III or IV drug-induced PAH

Macitentan (Opsumit®) is available under the PBS as an 'authority required' item for the treatment of:

  • WHO III or IV (iPAH) or anorexigen-induced PAH or hereditable PAH
  • WHO III or IV PAH secondary to connective tissue disease, and
  • WHO III or IV PAH associated with congenital systemic-to-pulmonary shunt including Eisenmenger's physiology

Sildenafil citrate (Revatio®) is available under the PBS as an 'authority required' item for the treatment of:

  • WHO functional Class III PPH, and
  • WHO functional Class III PAH secondary to connective tissue disease

Tadalafil (Adcirca®) is available under the PBS as an 'authority required' item for the treatment of:

  • WHO functional Class III PPH, and
  • WHO functional Class III PAH secondary to connective tissue disease

The Schedule of Pharmaceutical Benefits on the PBS website outlines the full list of restrictions.

These PAH agents are not PBS subsidised for patients with pulmonary hypertension secondary to interstitial lung disease associated with connective tissue disease, where the total lung capacity is less than 70% of that predicted.

Interchangeability between PAH agents is defined in the Schedule of Pharmaceutical Benefits on the PBS website.

PAH designated centres

Provider No. Name Address Suburb State Postcode
0030020L Alfred Hospital 75 Commercial Road MELBOURNE Vic 3004
0065020L Ashford Hospital
and Ashford Specialist Centre
57-59 Anzac Highway ASHFORD SA 5035
0030060F Austin Health 145 Studley Road HEIDELBERG Vic 3084
0030150B Box Hill Hospital 51 Nelson Road BOX HILL Vic 3128
0050230T Cairns Base Hospital The Esplanade CAIRNS Qld 4870
0090040A Calvary Public Hospital Cnr Belconnen Way & Haydon Drive BRUCE ACT 2617
0090030B Canberra Hospital Yamba Drive GARRAN ACT 2605
0010300T Children's Hospital at Westmead 176 Hawkesbury Road WESTMEAD NSW 2145
0010960A Coffs Harbour Health Campus 345 Pacific Highway COFFS HARBOUR NSW 2450
0012720A Concord Repatriation General Hospital Hospital Road CONCORD NSW 2139
0035260A Epworth Richmond 89 Bridge Road RICHMOND Vic 3121
71610H Fiona Stanley Hospital 102-118 Murdoch Drive MURDOCH  WA 6150
0060760W Flinders Medical Centre Flinders Drive BEDFORD PARK SA 5042
0030460K Frankston Hospital 2 Hastings Road FRANKSTON Vic 3199
0070010W Fremantle Hospital and Health Service Alma Street FREMANTLE WA 6160
0030470J Geelong Hospital Campus
Barwon Health
Bellarine Street GEELONG Vic 3221
0051903A Gold Coast University Hospital 1 Hospital Boulevard SOUTHPORT Qld 4215
0075490K Hollywood Private Hospital Monash Avenue NEDLANDS WA 6009
0012790J John Hunter Hospital
The Royal Newcastle Centre
Lookout Road NEW LAMBTON HEIGHTS NSW 2305
0056130T Lady Cilento Children's Hospital Raymond Terrace SOUTH BRISBANE Qld 4101
0011590B Lismore Base Hospital 60 Uralba Street LISMORE NSW 2480
0010200X Liverpool Hospital Elizabeth Street LIVERPOOL NSW 2170
0060680W Lyell McEwin Hospital Haydown Road ELIZABETH VALE SA 5112
0055360J Mater Hospital Pimlico 21-37 Fulham Road PIMLICO Qld 4810
0030970K Monash Medical Centre
Clayton Campus
246 Clayton Road CLAYTON Vic 3168
0075420B Mount Hospital 150 Mounts Bay Road PERTH WA 6000
016940A Port Macquarie Private Hospital Lake Road PORT MACQUARIE NSW 2444
0011860X Nepean Hospital Derby Street KINGSWOOD NSW 2747
0027100W Pendlebury Clinic Private Hospital 3/52 Pendlebury Road CARDIFF NSW 2285
0051400F Prince Charles Hospital Rode Road CHERMSIDE Qld 4032
0051080F Princess Alexandra Hospital 199 Ipswich Road WOOLLOONGABBA Qld 4102
0070020T Princess Margaret Hospital Roberts Road SUBIACO WA 6008
0060010K Queen Elizabeth Hospital 28 Woodville Road WOODVILLE SA 5011
0055110F Queensland Paediatric Cardiac Services
Mater Women's and Children's Private Health Services
Raymond Terrace SOUTH BRISBANE Qld 4101
0060730A Repatriation General Hospital Daws Road DAW PARK SA 5041
0060030H Royal Adelaide Hospital North Terrace ADELAIDE SA 5000
0050180J Royal Brisbane and Women's Hospital
James Mayne Building
Butterfield Street HERSTON Qld 4006
0031020F Royal Children's Hospital 50 Flemington Road PARKVILLE Vic 3052
0095070Y Royal Darwin Hospital Rocklands Drive TIWI NT 0810
0080060L Royal Hobart Hospital 48 Liverpool Street HOBART Tas 7000
0031030B Royal Melbourne Hospital
City Campus
300 Grattan Street PARKVILLE Vic 3052
0010310L Royal North Shore Hospital Pacific Highway ST LEONARDS NSW 2065
0070000X Royal Perth Hospital Wellington Street PERTH WA 6000
0010320K Royal Prince Alfred Hospital Missenden Road CAMPERDOWN NSW 2050
0070990J Sir Charles Gairdner Hospital Hospital Avenue NEDLANDS WA 6009
075240H St John of God Hospital 12 Salvado Road SUBIACO WA 6008
0010410J St George Hospital Gray Street KOGARAH NSW 2217
0031140X St Vincent's Hospital (Melbourne) Ltd 41 Victoria Parade FITZROY Vic 3065
0016150J St Vincent's Private Hospital 406 Victoria Street DARLINGHURST NSW 2010
016260B Sydney Adventist Hospital 185 Fox Valley Road WAHROONGA NSW 2076
0055740X Sunshine Coast Private Hospital Syd Lingard Drive BUDERIM Qld 4556
0012090T Tamworth Rural Referral Hospital 31 Dean Street TAMWORTH NSW, 2340
0051840B Townsville Hospital 100 Angus Smith Drive DOUGLAS Qld 4814
0055690L Wesley Hospital 451 Coronation Drive AUCHENFLOWER Qld 4066
0031850K Western Hospital 160 Gordon Street FOOTSCRAY Vic 3011
0012760T Westmead Hospital Cnr Hawkesbury Road & Darcy Road WESTMEAD NSW 2145
0060000L Women's & Children's Hospital 72 King William Road NORTH ADELAIDE SA 5006

Schedule item details

Dose - Ambrisentan (Volibris®) should be taken orally at a dose of 5 mg once daily. Additional benefit may be obtained by increasing the dose to 10 mg.  It is available in a pack size of 30 tablets.

Ambrisentan (Volibris®) is a category X medicine and must not be given to pregnant women. Pregnancy must be avoided during treatment and for at least 3 months following cessation of treatment with this medicine.

Children

There is no data available on the use of ambrisentan (Volibris®) in patients younger than 18 years, and therefore its use in this age group is not recommended.

Dose - Bosentan monohydrate (Tracleer®) is available in 62.5 mg tablets and 125 mg tablets (pack size is 60 tablets). No applications for increased maximum quantities will be authorised.

Bosentan monohydrate (Tracleer®) is a category X medicine and must not be given to pregnant women. Pregnancy must be avoided during treatment and for at least 3 months following cessation of treatment with this medicine.

The quantity approved will be based on the dosage recommendations in the Therapeutic Goods Administration (TGA)-approved product information.

Patient age and weight Bosentan monohydrate (Tracleer®) starting dose
first 4 weeks
Bosentan monohydrate (Tracleer®) maintenance dose
week 5 onwards
Adult > 40 kg 62.5 mg twice daily 125 mg twice daily
Patients 18 years or younger with a body weight > 40 kg
Adult < 40kg (12 years or older) 62.5 mg twice daily 62.5 mg twice daily
Patients 18 years or younger
with a body weight 20 to 40 kg
31.25 mg twice daily 62.5 mg twice daily
Patients 18 years or younger
with a body weight 10 to 20 kg
31.25 mg once daily 31.25 mg twice daily

Dose adjustment for patients with low body weight

The recommended initial and maintenance dose is 62.5 mg, twice daily, in patients who are 12 years or older and below 40 kg in weight.

Dose adjustment in children

There is limited experience with the use of bosentan (Tracleer®) in children. The recommended doses in children aged 3 years or over, based on the available information, are listed in the table above.

Dose - Epoprostenol sodium (Flolan®) is available as:

  • powder for intravenous infusion 500 micrograms (base) infusion administration set
  • powder for intravenous infusion 1.5 mg (base) infusion administration set

The quantity approved will be based on the dosage recommendations in the TGA-approved product information.

Dose - Iloprost trometamol (Ventavis®) is available as a nebuliser solution, 27.2 micrograms (equivalent to iloprost 20 micrograms per 2 mL).

The quantity approved will be based on the dosage recommendations in the TGA-approved product information.

Each inhalation session should start with 2.5 micrograms iloprost, as delivered at the mouthpiece of the inhalation device. The dose can be increased to 5.0 micrograms iloprost according to the individual need and tolerability.

The dose per inhalation session should be administered 6 to 9 times per day according to the individual need and tolerability.

Dose - Macitentan (Opsumit®) is available in 10 mg tablets (pack size is 30 tablets). This is a category X drug and must not be given to pregnant women. Pregnancy must be avoided during treatment and for at least 3 months following cessation of therapy.

The quantity approved will be based on the dosage recommendations in the TGA-approved product information.

Dose - Sildenafil citrate (Revatio®) is available in 20 mg tablets (pack size is 90 tablets). No applications for increased maximum quantities will be authorised.

The quantity approved will be based on the dosage recommendations in the TGA-approved product information.

Dose - Tadalafil (Adcirca®) is available in 20 mg tablets (pack size is 56 tablets). No applications for increased maximum quantities will be authorised.

The quantity approved will be based on the dosage recommendations in the TGA-approved product information.

Stopping treatment

Prescribers with patients who want to temporarily stop treatment with a PAH agent must submit a Pulmonary arterial hypertension Continuing authority application Supporting information form (PB071) for their current treatment within 1 month of stopping treatment.

Fax your completed form to us so it can be included in the patient's treatment history.

Lodging initial applications

All applications must be in writing and must include:

  • sufficient information to determine the patient's eligibility according to PBS criteria
  • results of a right heart catheterisation composite assessment
  • an echocardiography composite assessment, and
  • a 6 minute walk test

Details of prior vasodilator therapy must be provided for patients with WHO functional Class III with mean right arterial pressure (mRAP) less than or equal to 8 mm Hg.

Send all written applications to Complex Drugs Programs address on the contact us page.

Bosentan monohydrate

Pulmonary arterial hypertension Initial authority application Supporting information form (PB070) must include 2 written authority prescriptions—one for the first month of initiation therapy and another for the remaining 5 months of initial therapy.

For more information or to lodge an application for discontinuing treatment with Bosentan monohydrate (Tracleer®) contact us on the Complex Drugs Programs enquiry line.

Other listed PAH agents

Pulmonary arterial hypertension Initial authority application Supporting information form (PB070) must be in writing and must include an authority prescription for up to 6 months of therapy.

Lodging first continuing or changing applications

All applications must be in writing and must include:

  • sufficient information to determine the patient's eligibility according to PBS criteria
  • results of a right heart catheterisation composite assessment
  • an echocardiography composite assessment, and
  • a 6 minute walk test

A minimum of 2 test results must be provided to qualify for each continuation or change application where the patient has demonstrated stability or improvement of PPH or PAH relative to the baseline result.

A set of new baseline results must be provided for applications for patients who have not demonstrated stability or improvement of PPH or PAH relative to the baseline result, and who want to change to an alternative agent for which they are eligible.

Applications for Pulmonary arterial hypertension Continuing authority application Supporting information form (PB071) must be in writing and must include an authority prescription for up to 6 months of therapy.

Applications for change approvals for:

  • bosentan monohydrate (Tracleer®) must include 2 written authority prescriptions—1 for the first month of initiation therapy and another for the remaining 5 months of initial therapy, or
  • other listed PAH agents, which must include an authority prescription for up to 6 months of therapy

Send all written applications to Complex Drugs Programs address on the contact us page.

Subsequent continuing applications

If your patient has previously received PBS subsidised continuing treatment, subsequent applications for continuing treatment with that agent for the same condition may be made by contacting the Department of Human Services on 1800 700 270 (hours of operation: 8 am to 5 pm EST Monday to Friday) 

Written applications may be forwarded to Complex Drugs Programs address on the contact us page.

Lodging an application

For a patient starting initial PBS-subsidised treatment with a PAH agent lodge Pulmonary arterial hypertension Initial authority application Supporting information form (PB070) the patient and prescriber acknowledgements must be completed.

Lodge Pulmonary arterial hypertension Continuing authority application Supporting information form (PB071) for a patient’s application for:

  • the first continuing treatment
  • changing to an alternate PAH agent, or
  • demonstrating a response to treatment before stopping treatment

Further information

For more information contact the Complex Drugs Programs enquiry line.

Page last updated: 3 May 2016

This information was printed Sunday 31 July 2016 from humanservices.gov.au/health-professionals/enablers/primary-pulmonary-and-pulmonary-arterial-hypertension It may not include all of the relevant information on this topic. Please consider any relevant site notices at humanservices.gov.au/siteinformation when using this material.