Risk factors for chronic thromboembolic pulmonary hypertension

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

^* To whom correspondence should be addressed. E-mail: irene.lang{at}meduniwien.ac.at.

	Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by non-resolving pulmonary thromboemboli that can be treated by surgical pulmonary endarterectomy (PEA). We sought to confirm known and to identify novel CTEPH risk factors in a controlled retrospective cohort study of prevalent CTEPH cases collected in 3 European centers offering PEA.

Data from CTEPH patients were compared with non-thromboembolic precapillary pulmonary arterial hypertension cohorts at the participating institutions. The study population comprised 687 patients assessed at the time of diagnosis between 1996 and 2007. VA-shunts and infected pacemakers (odds ratio (OR) and 95% confidence interval, 76.40 [7.67–10351], p<0.001), splenectomy (OR 17.87 [1.56–2438], p=0.017), previous venous thromboembolism (VTE) (OR 4.52 [2.35–9.12], p<0.001), recurrent VTE (OR 14.49 [5.40–43.08], p<0.001), blood groups non-0 (OR (OR 2.09 [1.12–3.94], p=0.019), and lupus anticoagulant/anti-phospholipid antibodies (OR 4.20 [1.56–12.21], p=0.004) were more often associated with CTEPH. Thyroid replacement therapy (OR 6.10 [2.73–15.05], p<0.001) and a history of malignancy (OR 3.76 [1.47–10.43], p=0.005) emerged as novel CTEPH risk factors.

This European database confirmed previous knowledge on CTEPH risk factors, and identified thyroid replacement therapy and a history of malignancy as new medical conditions associated with CTEPH.