Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany

Clin Res Cardiol. 2018 Jul;107(7):548-553. doi: 10.1007/s00392-018-1215-5. Epub 2018 Feb 15.


Background: The incidence of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. Previous studies from the United Kingdom and Spain have reported incidence rates of 1.75 and 0.9 per million, respectively. These figures, however, may underestimate the true incidence of CTEPH.

Methods: We prospectively enrolled patients newly diagnosed with CTEPH within 2016 in Germany. Data were obtained from the three German referral centers and from the German branch of COMPERA, a European pulmonary hypertension registry. The CTEPH incidence was calculated based on German population data, and patient characteristics and treatment patterns were described.

Results: A total of 392 patients were newly diagnosed with CTEPH within 2016 in Germany, yielding an incidence of 5.7 new cases per million adults. The (mean ± standard deviation) age was 63.5 ± 15.0 years; males and females were equally affected; 76.3% of the patients had a history of venous thromboembolism. A total of 197 (50.3%) patients underwent pulmonary endarterectomy. Almost all non-operated patients received targeted drug therapy, and 49 patients (25.1% of the non-operated patients) were treated with balloon pulmonary angioplasty.

Conclusion: The incidence of CTEPH in Germany 2016 was 5.7 per million adults and thus higher than previously reported from other countries. Half of the patients were operated while the remaining patients received medical or interventional therapies.

Clinical trials registration: NCT02660463 and NCT01347216.

Keywords: Balloon pulmonary angioplasty; Chronic thromboembolic pulmonary hypertension; Epidemiology; Incidence; Pulmonary endarterectomy.

Publication types

  • Multicenter Study

MeSH terms

  • Aged
  • Angioplasty, Balloon
  • Antihypertensive Agents / therapeutic use
  • Chronic Disease
  • Endarterectomy
  • Female
  • Germany / epidemiology
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / epidemiology*
  • Hypertension, Pulmonary / physiopathology
  • Hypertension, Pulmonary / therapy
  • Incidence
  • Male
  • Middle Aged
  • Prospective Studies
  • Pulmonary Embolism / diagnosis
  • Pulmonary Embolism / epidemiology*
  • Pulmonary Embolism / physiopathology
  • Pulmonary Embolism / therapy
  • Registries
  • Risk Factors
  • Time Factors
  • Treatment Outcome
  • Venous Thromboembolism / diagnosis
  • Venous Thromboembolism / epidemiology*
  • Venous Thromboembolism / physiopathology
  • Venous Thromboembolism / therapy


  • Antihypertensive Agents

Associated data