• Thorsten Kramm, Heinrike Wilkens, Jan Fuge, Hans-Joachim Schäfers, Stefan Guth, Christoph B Wiedenroth, Bettina Weingard, Doerte Huscher, David Pittrow, Serghei Cebotari, Marius M Hoeper, Eckhard Mayer, and Karen M Olsson.
• Kerckhoff Heart and Lung Center, Bad Nauheim, Germany.
• Clin Res Cardiol. 2018 Jul 1; 107 (7): 548-553.

BackgroundThe 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.MethodsWe 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.ResultsA 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.ConclusionThe 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 Registrationhttp://www.clinicaltrials.gov NCT02660463 and NCT01347216.

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