Management and outcomes in chronic thromboembolic pulmonary hypertension: From expert centers to a nationwide perspective.

Autor: Escribano-Subías P; Pulmonary Hypertension Unit, RIC group, Cardiology Department, Hospital Universitario 12 de Octubre, Madrid, Spain. Electronic address: pilar.escribano.subias@gmail.com., Del Pozo R; Pulmonary Hypertension Unit, Pneumonology Department, Hospital Universitario 12 de Octubre, Madrid, Spain., Román-Broto A; Department of Pneumonology, Hospital Universitari Vall d´Hebrón, Universitat Autonoma, Barcelona, Spain; Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Spain., Domingo Morera JA; Department of Pneumonology, Hospital Universitario Miguel Servet, Zaragoza, Spain., Lara-Padrón A; Department of Cardiology, Hospital Universitario de Canarias, Santa Cruz de Tenerife, Spain., Elías Hernández T; Department of Pneumonology, Hospital Universitario Virgen del Rocío, Sevilla, Spain., Molina-Ferragut L; Department of Cardiology, Hospital del Mar, Barcelona, Spain; Heart Diseases Biomedical Research Group, IMIM (Hospital del Mar Medical Research Institute), Barcelona, Spain., Blanco I; Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Spain; Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Spain., Cortina J; Pulmonary Hypertension Unit, Cardiac Surgery Department, Hospital Universitario 12 de Octubre, Madrid, Spain., Barberà JA; Biomedical Research Networking Center on Respiratory Diseases (CIBERES), Spain; Department of Pulmonary Medicine, Hospital Clínic-IDIBAPS, University of Barcelona, Barcelona, Spain.
Jazyk: angličtina
Zdroj: International journal of cardiology [Int J Cardiol] 2016 Jan 15; Vol. 203, pp. 938-44. Date of Electronic Publication: 2015 Nov 10.
DOI: 10.1016/j.ijcard.2015.11.039
Abstrakt: Background: The Spanish "Registry of Pulmonary Arterial Hypertension" (REHAP), started in 2007, includes chronic thromboembolic hypertension (CTEPH) patients. Based on data provided by this registry and retrospective data from patients diagnosed during 2006 (≤ 12 months since the registry was created), clinical management and long-term outcomes of CTEPH patients are analyzed nationwide for the first time in a scenario of a decentralized organization model of CTEPH management.
Methods and Results: A total of 391 patients (median [Q1:Q3] age 63.7 [48.0;73.3] years, 58% females) with CTEPH included during the period January 1, 2006-December 31, 2013 in the REHAP registry were analyzed. Rate of pulmonary endarterectomy (PEA) was 31.2%, and highly asymmetric among centers: rate was 47.9% at two centers designated as CTEPH expert centers, while it was 4.6% in other centers. Among patients not undergoing PEA, 82% were treated with therapies licensed for pulmonary arterial hypertension (PAH). Five-year survival rate was 86.3% for PEA patients, and 64.9% for non-PEA patients. Among non-PEA patients, presenting proximal lesions (42% of non-referred patients) was associated with a 3-fold increase in mortality. PEA patients achieved significantly better hemodynamic and clinical outcomes at one-year follow-up compared to non-PEA patients. Patients not being referred for PEA assessment were older and had a worse functional capacity. Older age was the most deterrent factor for non-operability.
Conclusion: Despite the increase in diagnosis and expertise in PEA-specialized centers, an important percentage of patients do not benefit of PEA in a decentralized organization model of CTEPH management.
(Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)
Databáze: MEDLINE
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