Last edited 08/2022

  • Poor median survival rates in untreated patients - 2.8 years from diagnosis (1):
    • previous 5 year survival was 40% but this has been improved with advances in medical therapy
  • Chronic thromboembolic pulmonary hypertension (CTEPH):
    • majority of patients can be cured with normalisation of pulmonary pressure following pulmonary endarterectomy surgery with improvement in symptoms and prognosis, with a > 90% 5 year survival
  • In some patients with pulmonary hypertension, bilateral lung or heart lung transplantation may be considered. The 5 year survival following transplantation is approximately 50-60%.
  • Development of cor pulmonale as a result of a primary pulmonary disease usually heralds a poorer prognosis (2).