Risk stratification in chronic thromboembolic pulmonary hypertension predicts survival

Research output: Contribution to journalArticle


Objectives. To investigate if the pulmonary arterial hypertension (PAH) risk assessment tool presented in the 2015 ESC/ERS guidelines is valid for patients with chronic thromboembolic pulmonary hypertension (CTEPH) when taking pulmonary endarterectomy (PEA) into account. Design. Incident CTEPH patients registered in the Swedish PAH Registry (SPAHR) between 2008 and 2016 were included. Risk stratification performed at baseline and follow-up classified the patients as low-, intermediate-, or high-risk using the proposed ESC/ERS risk algorithm. Results. There were 250 CTEPH patients with median age (interquartile range) 70 (14) years, and 53% were male. Thirty-two percent underwent PEA within 5 (6) months. In a multivariable model adjusting for age, sex, and pharmacological treatment, patients with intermediate-risk or high-risk profiles at baseline displayed an increased mortality risk (Hazard Ratio [95% confidence interval]: 1.64 [0.69–3.90] and 5.39 [2.13–13.59], respectively) compared to those with a low-risk profile, whereas PEA was associated with better survival (0.38 [0.18–0.82]). Similar impact of risk profile and PEA was seen at follow-up. Conclusion. The ESC/ERS risk assessment tool identifies CTEPH patients with reduced survival. Furthermore, PEA improves survival markedly independently of risk group and age. Take home message: The ESC/ERS risk stratification for PAH predicts survival also in CTEPH patients, even when taking PEA into account.


External organisations
  • Umeå University
  • Linköping University
  • Sahlgrenska University Hospital
  • Karolinska University Hospital
  • Skåne University Hospital
  • Uppsala University Hospital
  • Karolinska Institutet
  • University of Gothenburg
  • Uppsala University
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Public Health, Global Health, Social Medicine and Epidemiology
  • Cardiac and Cardiovascular Systems


  • CTEPH, goal-oriented treatment, pulmonary endarterectomy, risk stratification, survival
Original languageEnglish
JournalScandinavian Cardiovascular Journal
Publication statusE-pub ahead of print - 2020 Jun 26
Publication categoryResearch