Prognosis

• 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).

References:

• (1) D'Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991;115: 343-349.
• (2) Garrison DM, Pendela VS, Memon J. Cor Pulmonale. [Updated 2021 Aug 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK430739/ (Accessed on 15 Aug 2022).