Chronic thromboembolic pulmonary hypertension in Spain: a decade of change

Paula Martínez-Santosa, María Teresa Velázquez-Martínb, Joan Albert Barberác,d, Cristina Fernández Péreze, Manuel López-Meseguerf,g, Raquel López-Reyesh, Amaya Martínez-Meñacai, Antonio Lara-Padrónj, Juan Antonio Domingo-Morerad, Isabel Blancoc,g, Pilar Escribano-Subíasb,k,, on behalf of REHAP investigators

Servicio de Cardiología, Hospital Universitario Puerta de Hierro Majadahonda, Majadahonda, Madrid, SpainUnidad de Hipertensión Pulmonar, Servicio de Cardiología, Hospital Universitario 12 de Octubre, Madrid, SpainServicio de Neumología, Hospital Clínic, Instituto de Investigaciones Biomédicas August Pi i Sunyer (IDIBAPS), Universidad de Barcelona, Barcelona, SpainServicio de Neumología, Hospital Universitario Miguel Servet, Zaragoza, SpainServicio de Medicina Preventiva, Hospital Universitario San Carlos, Madrid, SpainServicio de Neumología, Hospital Universitario Vall d’Hebron, Barcelona, SpainCentro de Investigación Biomédica en Red Enfermedades Respiratorias (CIBERES), Madrid, SpainServicio de Neumología, Hospital Universitario La Fe, Valencia, SpainServicio de Neumología, Hospital Universitario Marqués de Valdecilla, Santander, Cantabria, SpainServicio de Cardiología, Hospital Universitario de Canarias, Sta. Cruz de Tenerife, SpainCentro de Investigación Biomédica en Red Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain

Received 2 December 2019, Accepted 25 May 2020, Available online 9 July 2020.


Background: Chronic thromboembolic pulmonary hypertension (CTEPH) treatment has evolved over the last decade. Increasing evidence regarding new therapeutic developments has shown clinical benefit among these patients in different scenarios. However, there is scarce information about the long-term impact of these achievements in a real-life population on a national scale. We aimed to analyze the evolution of CTEPH assistance in Spain from 2007 to 2017.

Methods: We prospectively collected epidemiological, clinical and prognostic data from CTEPH patients consecutively included in the Spanish REHAP registry from January 1, 2007, to December 31, 2017. We evaluated differences across time, establishing 2013 as the referral date for analysis. Clinical and all-cause mortality data were gathered during this period.  

Results: Eight hundred thirteen patients were included. The CTEPH reported incidence in Spain increased from 1.02 in 2007 to 2.4 per million individuals in 2017. Overall, 245 (30.1%) patients were selected for surgery and 52 (6.4%) for percutaneous treatment. Out of the 813 patients, 537 (66%) were referred to an expert PH-center. Baseline functional capacity and the need for treatment with prostacyclin analogs associated a worse prognosis. Likewise, patients diagnosed between 2007 and 2012 died more frequently than those diagnosed 2013 onward (HR 6.06; CI 95% 4.04-9.10, p<0.0001). Survival rates of patients who underwent an invasive procedure (pulmonary thrombendarterectomy or balloon pulmonary angioplasty) were remarkably high (87.1% and 94.3% at 5 years, respectively).

Conclusions Over the last decade, CTEPH diagnosis and prognosis have consistently improved. Baseline disease severity determined the risk profile. Patients who underwent pulmonary thrombendarterectomy or balloon pulmonary angioplasty associated with a better outcome. 

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