Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are two of the key subgroups of pulmonary hypertension.

Pulmonary hypertension is caused by high blood pressure in the pulmonary arteries, which carry blood from the heart to the lungs. Both PAH and CTEPH can damage the right part of the heart making it unable to efficiently circulate blood around the body. It can lead to heart failure and can be fatal.

“PAH and CTEPH are rare cardiopulmonary diseases and patients should be treated in expert centres. In recent years, novel therapies for PAH and CTEPH have improved not only the quality of life and symptoms but also morbidity and mortality. It’s vital that we collect data on these conditions at a national and international level so we can understand the course of the disease and how different treatments are working.” Mona Lichtblau, Early Career Representative for the European Respiratory Society’s Pulmonary vascular diseases assembly and Pulmonologist at the Dept of Pneumology, University Hospital Zürich, Zürich, Switzerland.

The charts below present the data available for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Data was taken primarily from a systematic review that included incidence and prevalence of people with PAH or CTEPH. It reported estimates for adults and children separately.  Incidence and prevalence were reported for Poland (PAH), Latvia (PAH & CTEPH), Germany (PAH) and Sweden (PAH & CTEPH). Only incidence or prevalence were reported for Slovakia (prevalence of CTEPH), France (prevalence of CTEPH), Germany (incidence of CTEPH), Portugal (incidence of PAH and CTEPH), Spain (incidence of PAH & CTEPH).

A second review modelled incidence and prevalence of CTEPH for five countries in Europe: France, Germany, Spain, Italy, UK. Only data for Italy was used from this study as more recent estimates were available for the other countries.

Three further studies reported data for the Netherlands, Czech Republic and the UK.

Methods of data collection and analysis varied across studies and therefore the comparability of estimates produced by various methods is unclear.

Studies used:

  1. Leber L, Beaudet A, Muller A. Epidemiology of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: identification of the most accurate estimates from a systematic literature review. Pulmonary Circulation 2021; 11: 2045894020977300.
  2. Gall H, Hoeper MM, Richter MJ, Cacheris W, Hinzmann B, Mayer E. An epidemiological analysis of the burden of chronic thromboembolic pulmonary hypertension in the USA, Europe and Japan. Eur Respir Rev 2017; 26: 31.
  3. Jansa P, Ambroz D, Kuhn M, et al. Epidemiology of chronic thromboembolic pulmonary hypertension (CTEPH) in the Czech Republic. Pulmonary Circulation 2022; 12: e12038.
  4. Moreira EM, Gall H, Leening MJ, et al. Prevalence of Pulmonary Hypertension in the General Population: The Rotterdam Study. PLoS ONE 2015; 10: e0130072.
  5. digital N. National Audit of Pulmonary Hypertension Great Britain, 2020-21; 20th January 2022.

All charts are freely available to download by clicking the three dots in the top right-hand corner. Please reference Lung Facts when sharing.

  • CTEPH prevalence rate
  • CTEPH incidence rate
  • PAH prevalence rate
  • PAH incidence rate