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Thromb Haemost 2013; 110(01): 83-91
DOI: 10.1160/TH13-02-0097
Blood Coagulation, Fibrinolysis and Cellular Haemostasis
Schattauer GmbH

Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension

A case-control study
Irene M. Lang
1   Medical University of Vienna, Austria
,
Gérald Simonneau
2   University Paris Sud (Paris XI), INSERM U 999, Hôpital Bicêtre, Le Kremlin Bicêtre, France
,
Joanna W. Pepke-Zaba
3   Papworth Hospital, Cambridge, UK
,
Eckhard Mayer
4   Kerckhoff Clinic Heart and Lung Centre, Bad Nauheim, Germany
,
David Ambrož
5   Clinical Department of Cardiology and Angiology of the First Faculty of Medicine and General Teaching Hospital, Prague, Czech Republic
,
Isabel Blanco
6   Hospital Clínic–Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS)-CIBER Enfermedades Respiratorias, Universtiy of Barcelona, Spain
,
Adam Torbicki
7   Medical Centre of Postgraduate Education, ECZ, Otwock, Poland
,
Sören Mellemkjaer
8   Aarhus University Hospital, Skejby, Aarhus, Denmark
,
Azzedine Yaici
2   University Paris Sud (Paris XI), INSERM U 999, Hôpital Bicêtre, Le Kremlin Bicêtre, France
,
Marion Delcroix
9   University Hospital Gasthuisberg, Leuven, Belgium
› Author AffiliationsFinancial support: The CTEPH registry is supported by a research grant from Actelion Pharmaceuticals Ltd.
Further Information

Publication History

Received: 04 February 2013

Accepted after major revision: 04 April 2013

Publication Date:
30 November 2017 (online)

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Summary

Chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary hypertension (IPAH) share a similar clinical presentation, and a differential diagnosis requires a thorough workup. Once CTEPH is confirmed, patients who can be safely operated have to be identified. We investigated risk factors associated with CTEPH and IPAH, and the criteria for the selection of operable CTEPH patients. This case-control study included 436 consecutive patients with CTEPH and 158 with IPAH in eight European centres, between 2006 and 2010. Conditions identified as risk factors for CTEPH included history of acute venous thromboembolism (p < 0.0001), large size of previous pulmonary embolism (p = 0.0040 in univariate analysis), blood groups non-O (p < 0.0001 in univariate analysis), and older age (p = 0.0198), whereas diabetes mellitus (p = 0.0006), female gender (p = 0.0197) and higher mean pulmonary artery pressure (p = 0.0103) were associated with increased likelihood for an IPAH diagnosis. Operability of CTEPH patients was associated with younger age (p = 0.0108), proximal lesions (p ≤ 0.0001), and pulmonary vascular resistance below 1200 dyn.s.cm-5 (p = 0.0080). Non-operable CTEPH patients tended to be less differentiable from IPAH patients by risk factor analysis than operable patients. This study confirmed the association of CTEPH with history of acute venous thromboembolism and blood groups non-O, and identified diabetes mellitus and higher mean pulmonary artery pressure as factors suggesting an IPAH diagnosis. Non-operable CTEPH is more similar to IPAH than operable CTEPH regarding risk factors.

Keywords

Chronic lung disease - endarterectomy - pulmonary arterial hypertension - risk factor
 

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