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BioMed Research International
Volume 2013 (2013), Article ID 489574, 8 pages
http://dx.doi.org/10.1155/2013/489574
Clinical Study

Pulmonary Hypertension in Portugal: First Data from a Nationwide Registry

1Department of Cardiology, Hospitais da Universidade de Coimbra, Centro Hospitalar e Universitário de Coimbra, Praceta Mota Pinto, 3000 Coimbra, Portugal
2Institute for Biomedical Imaging and Life Sciences, Faculty of Medicine of University of Coimbra, Azinhaga de Santa Comba, 3000 Coimbra, Portugal
3Department of Internal Medicine, Hospital Geral de Santo António, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099 Porto, Portugal
4Department of Cardiology, Hospital Santa Marta, Centro Hospitalar de Lisboa Central, Rua de Santa Marta, 1169 Lisboa, Portugal
5Department of Pneumology, Hospital Santos Silva, Centro Hospitalar Gaia/Espinho, Rua Conceição Fernandes, 4434 Vila Nova de Gaia, Portugal
6Department of Cardiology, Hospital de Santa Maria, Centro Hospitalar de Lisboa Norte, Avenida Professor Egas Moniz, 1649 Lisboa, Portugal

Received 8 April 2013; Accepted 26 August 2013

Academic Editor: Zhirong Sun

Copyright © 2013 Rui Baptista et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Introduction. Pulmonary arterial hypertension (PAH) is a rare disease that must be managed in specialized centers; therefore, the availability of epidemiological national data is critical. Methods. We conducted a prospective, observational, and multicenter registry with a joint collaboration from five centers from Portugal and included adult incident patients with PAH or chronic thromboembolic pulmonary hypertension (CTEPH). Results. Of the 79 patients enrolled in this study, 46 (58.2%) were classified as PAH and 33 patients (41.8%) as CTEPH. PAH patients had a mean age of 43.4 ± 16.4 years. Idiopathic PAH was the most common etiology (37%). At presentation, PAH patients had elevated right atrial pressure (RAP) (7.7 ± 5.9 mmHg) and mean pulmonary vascular resistance (11.4 ± 6.5 Wood units), with a low cardiac index (2.7 ± 1.1 L·min−1·m−2); no patient was under selective pulmonary vasodilators; however, at follow-up, most patients were on single (50%), double (28%), or triple (9%) combination vasodilator therapy. One-year survival was 93.5%, similar to CTEPH patients (93.9%), that were older (60.0 ± 12.5 years) and had higher RAP (11.0 ± 5.2 mmHg, ). Conclusions. We describe for the first time nationwide data on the diagnosis, management, and prognosis of PAH and CTEPH patients in Portugal. Clinical presentation and outcomes are comparable with those reported on other national registries.