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Published online before print September 17, 2008, 10.1183/09031936.00087608
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Eur Respir J 2009; 33:325-331
Copyright ©ERS Journals Ltd 2009

Risk factors for chronic thromboembolic pulmonary hypertension

D. Bonderman1, H. Wilkens2, S. Wakounig3, H-J. Schäfers4, P. Jansa5, J. Lindner6, I. Simkova7, A. M. Martischnig1, J. Dudczak1, R. Sadushi1, N. Skoro-Sajer1, W. Klepetko8 and I. M. Lang1

1 Division of Cardiology, 3 Core Unit for Medical Statistics and Informatics, 8 Division of Cardiothoracic Surgery, Vienna General Hospital, Medical University of Vienna, Vienna, Austria, 7 Cardiology Clinic, National Institute of Cardiovascular Diseases, Dept of Cardiology, Slovak Medical University, Bratislava, Slovak Republic, 2 Depts of Pneumology, Allergology, and Environmental Medicine, 4 Thoracic and Cardiovascular Surgery, University Hospital of Saarland, Homburg/Saar, Germany, 5 2nd Medical Dept, Clinical Dept of Cardiology and Angiology, and 6 2nd Surgical Dept, Clinical Dept of Cardiovascular Surgery, General Teaching Hospital, Prague, Czech Republic.

CORRESPONDENCE: I. M. Lang, Dept of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria. Fax: 43 1404004216. E-mail: irene.lang{at}meduniwien.ac.at

Keywords: Pulmonary hypertension, risk factors, thromboembolic disease

Received: June 9, 2008
Accepted September 1, 2008

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterised by nonresolving pulmonary thromboemboli that can be treated by surgical pulmonary endarterectomy (PEA). The authors of the present study sought to confirm known and to identify novel CTEPH risk factors in a controlled retrospective cohort study of prevalent CTEPH cases collected in three European centres offering PEA.

Data from CTEPH patients were compared with nonthromboembolic pre-capillary pulmonary arterial hypertension cohorts at the participating institutions.

The study population comprised 687 patients assessed at the time of diagnosis between 1996 and 2007. Ventriculo-atrial shunts and infected pacemakers (odds ratio (OR) 76.40, 95% confidence interval (CI) 7.67–10,351), splenectomy (OR 17.87, 95% CI 1.56–2,438), previous venous thromboembolism (VTE; OR 4.52, 95% CI 2.35–9.12), recurrent VTE (OR 14.49, 95% CI 5.40–43.08), blood groups other than 0 (2.09, 95% CI 1.12–3.94), and lupus anticoagulant/antiphospholipid antibodies (OR 4.20, 95% CI 1.56–12.21) were more often associated with CTEPH. Thyroid replacement therapy (OR 6.10, 95% CI 2.73–15.05) and a history of malignancy (OR 3.76, 95% CI 1.47–10.43) emerged as novel CTEPH risk factors.

In conclusion, the European database study confirmed previous knowledge of chronic thromboembolic pulmonary hypertension risk factors, and identified thyroid replacement therapy and a history of malignancy as new medical conditions associated with chronic thromboembolic pulmonary hypertension.




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