Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry
Joanna Pepke-Zaba, Marion Delcroix, Irene Lang, Eckhard Mayer, Pavel Jansa, David Ambroz, Carmen Treacy, Andrea M D'Armini, Marco Morsolini, Repke Snijder, Paul Bresser, Adam Torbicki, Bent Kristensen, Jerzy Lewczuk, Iveta Simkova, Joan A Barberà, Marc de Perrot, Marius M Hoeper, Sean Gaine, Rudolf Speich, Miguel A Gomez-Sanchez, Gabor Kovacs, Abdul Monem Hamid, Xavier Jaïs, Gérald Simonneau, Joanna Pepke-Zaba, Marion Delcroix, Irene Lang, Eckhard Mayer, Pavel Jansa, David Ambroz, Carmen Treacy, Andrea M D'Armini, Marco Morsolini, Repke Snijder, Paul Bresser, Adam Torbicki, Bent Kristensen, Jerzy Lewczuk, Iveta Simkova, Joan A Barberà, Marc de Perrot, Marius M Hoeper, Sean Gaine, Rudolf Speich, Miguel A Gomez-Sanchez, Gabor Kovacs, Abdul Monem Hamid, Xavier Jaïs, Gérald Simonneau
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.
Source: PubMed