Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany

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, Karen M Olsson, 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, Karen M Olsson

Abstract

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: http://www.clinicaltrials.gov NCT02660463 and NCT01347216.

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

Conflict of interest statement

T. Kramm has received speaker fees from Actelion, Bayer and GSK. H. Wilkens has received speaker fees and honoraria for consultation from Actelion, Bayer, Boehringer-Ingelheim, GSK, MSD, Pfizer and Roche. J. Fuge has nothing to disclose. HJ Schäfers has received speaker fees from Bayer. S. Guth has received speaker fees from Actelion, Bayer, GSK and Pfizer. CB Wiedenroth has received speaker fees or consultant honoraria from Actelion, Bayer AG, BTG, MSD and Pfizer. B. Weingard has received speaker fees and consultation honoraria from Bayer. D. Huscher has received author and consultation honoraria from Actelion. D. Pittrow has received speaker fees or honoraria for consultations from Actelion, AstraZeneca, Aspen, Bayer, Boehringer Ingelheim, Daiichi Sankyo, Novartis, Shield and Pfizer. M.M. Hoeper has received speaker fees and honoraria for consultations from Actelion, Bayer, Gilead, GSK, MSD and Pfizer. S. Cebotari has nothing to disclose. E. Mayer has received speaker and consulting fees from Actelion, Bayer, GSK, and Pfizer. KM Olsson has received speaker fees and honoraria for consultations from Actelion, Bayer, GSK, Pfizer and United Therapeutics.

Figures

Fig. 1
Fig. 1
Treatment patterns in 392 patients with newly diagnosed chronic thromboembolic pulmonary hypertension (CTEPH). PH pulmonary hypertension, BPA balloon pulmonary angioplasty

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Source: PubMed

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