Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Author:

Pepke-Zaba Joanna1,Delcroix Marion1,Lang Irene1,Mayer Eckhard1,Jansa Pavel1,Ambroz David1,Treacy Carmen1,D'Armini Andrea M.1,Morsolini Marco1,Snijder Repke1,Bresser Paul1,Torbicki Adam1,Kristensen Bent1,Lewczuk Jerzy1,Simkova Iveta1,Barberà Joan A.1,de Perrot Marc1,Hoeper Marius M.1,Gaine Sean1,Speich Rudolf1,Gomez-Sanchez Miguel A.1,Kovacs Gabor1,Hamid Abdul Monem1,Jaïs Xavier1,Simonneau Gérald1

Affiliation:

1. From the Papworth Hospital, Cambridge, United Kingdom (J.P.-Z., C.T.); University Hospital Gasthuisberg, Leuven, Belgium (M.D.); Medical University of Vienna, Vienna, Austria (I.L.); Kerckhoff Heart and Lung Center, Bad Nauheim, Germany (E.M.); Clinical Department of Cardiology and Angiology of the First Faculty of Medicine and General Teaching Hospital, Prague, Czech Republic (P.J., D.A.); San Matteo Hospital, University of Pavia, Pavia, Italy (A.M.D., M.M.); St. Antonius Ziekenhuis, Nieuwegein,...

Abstract

Background— Chronic thromboembolic pulmonary hypertension (CTEPH) is often a sequel of venous thromboembolism with fatal natural history; however, many cases can be cured by pulmonary endarterectomy. The clinical characteristics and current management of patients enrolled in an international CTEPH registry was investigated. Methods and Results— The international registry included 679 newly diagnosed (≤6 months) consecutive patients with CTEPH, from February 2007 until January 2009. Diagnosis was confirmed by right heart catheterization, ventilation-perfusion lung scintigraphy, computerized tomography, and/or pulmonary angiography. At diagnosis, a median of 14.1 months had passed since first symptoms; 427 patients (62.9%) were considered operable, 247 (36.4%) nonoperable, and 5 (0.7%) had no operability data; 386 patients (56.8%, ranging from 12.0%– 60.9% across countries) underwent surgery. Operable patients did not differ from nonoperable patients relative to symptoms, New York Heart Association class, and hemodynamics. A history of acute pulmonary embolism was reported for 74.8% of patients (77.5% operable, 70.0% nonoperable). Associated conditions included thrombophilic disorder in 31.9% (37.1% operable, 23.5% nonoperable) and splenectomy in 3.4% of patients (1.9% operable, 5.7% nonoperable). At the time of CTEPH diagnosis, 37.7% of patients initiated at least 1 pulmonary arterial hypertension–targeted therapy (28.3% operable, 53.8% nonoperable). Pulmonary endarterectomy was performed with a 4.7% documented mortality rate. Conclusions— Despite similarities in clinical presentation, operable and nonoperable CTEPH patients may have distinct associated medical conditions. Operability rates vary considerably across countries, and a substantial number of patients (operable and nonoperable) receive off-label pulmonary arterial hypertension–targeted treatments.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Physiology (medical),Cardiology and Cardiovascular Medicine

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