04 February 2019 by Lucilla Piccari

Preliminary results of a national registry of pulmonary hypertension associated with respiratory diseases


Pulmonary hypertension (PH) is a common complication in chronic lung diseases associated with higher mortality and poor clinical course. However, the prevalence and characteristics of this relevant complication are still largely unknown. The Spanish Registry for PH associated with respiratory diseases (REHAR) was created to define the clinical and epidemiological characteristics of these patients. Here we present the characteristics of the first patients included in the registry.


The REHAR registry is a web-based platform created in 2016. Patients were registered from 9 PH expert units, between September 2017-November 2018. Anthropometric and functional parameters, arterial and mixed venous respiratory gases, pulmonary hemodynamics and PH treatment data were analysed according to the underlying diagnosis.
Results: 98 of 101 patients with complete data were analysed. Results are shown below.


Values are mean ± SD. (#) p < 0.05 compared to COPD group; (*) p <0.05 compared to ILD group. 1Severe PH assessed according to guidelines’ criteria (Galié, N. et al, Eur Respir J, 34(6), 1219–1263).

There were no differences in hemodynamic, functional status or gas exchange, except for the expected differences in pulmonary function test profiles and a lower distance in the 6-min walk test for patients with COPD, compared to those with CPFE. Most patients did not receive targeted PH therapy; those who did were mainly from the severe PH group. The most commonly used drugs were phosphodiesterase-5 inhibitors (iPDE5, 17%), followed by endothelin-receptor antagonists (6%).


the first results from the Spanish Registry of PH in respiratory diseases show that most patients included suffered from severe PH, likely due to referral bias since they would have been referred to expert PH centres for right heart catheterization. PH treatment associated with respiratory diseases is restricted to those with severe PH and mainly focused on the use of iPDE5.


Funded by CIBERES (2016), with the endorsement of SEPAR.

About the author

profile picture of Lucilla Piccari

Lucilla Piccari

Medical Assistant

Hospital del Mar


Key Contributors

Lucilla Piccari 1,2, Isabel Blanco 2, Manuel López Mesegue r3, Roberto del Pozo 4, Xavier Pomares 5, Salud Santos 6, Javier de Miguel 7, Gustavo Juan 8, Mª José Rodríguez 9, Ernest Sala 10, Luis Jara 11, Juan Antonio Rodríguez Morera 12 Joan Albert Barberà 2, Diego A. Rodríguez 1 : 1.Hospital del Mar-IMIM (Barcelona); 2.Hospital Clínic-IDIBAPS (Barcelona); 3.Hospital Vall d’Hebrón (Barcelona); 4.Hospital Juan Ramón Jiménez (Huelva); 5.Corporación Sanitaria Parc Taulí-Hospital de Sabadell; 6.Hospital de Bellvitge (Barcelona); 7.Hospital Gregorio Marañon (Madrid); 8Hospital general de Valencia (Valencia); 9.Fundación Jiménez Díaz (Madrid); 10.Hospital Son Espases (Palma de Mallorca); 11.Hospital Virgen del Rocío (Sevilla); 12 .Hospital Miquel Servet (Zaragoza).

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