Pulmonary Hypertension in Portugal: First Data from a Nationwide Registry
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 fi...
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doaj-ba8ffb2709c3414fbff4dc42ad7612b62020-11-24T22:52:35ZengHindawi LimitedBioMed Research International2314-61332314-61412013-01-01201310.1155/2013/489574489574Pulmonary Hypertension in Portugal: First Data from a Nationwide RegistryRui Baptista0José Meireles1Ana Agapito2Graça Castro3António Marinho da Silva4Teresa Shiang5Fabienne Gonçalves6Susana Robalo-Martins7António Nunes-Diogo8Abílio Reis9Department of Cardiology, Hospitais da Universidade de Coimbra, Centro Hospitalar e Universitário de Coimbra, Praceta Mota Pinto, 3000 Coimbra, PortugalDepartment of Internal Medicine, Hospital Geral de Santo António, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099 Porto, PortugalDepartment of Cardiology, Hospital Santa Marta, Centro Hospitalar de Lisboa Central, Rua de Santa Marta, 1169 Lisboa, PortugalDepartment of Cardiology, Hospitais da Universidade de Coimbra, Centro Hospitalar e Universitário de Coimbra, Praceta Mota Pinto, 3000 Coimbra, PortugalDepartment of Cardiology, Hospitais da Universidade de Coimbra, Centro Hospitalar e Universitário de Coimbra, Praceta Mota Pinto, 3000 Coimbra, PortugalDepartment of Pneumology, Hospital Santos Silva, Centro Hospitalar Gaia/Espinho, Rua Conceição Fernandes, 4434 Vila Nova de Gaia, PortugalDepartment of Internal Medicine, Hospital Geral de Santo António, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099 Porto, PortugalDepartment of Cardiology, Hospital de Santa Maria, Centro Hospitalar de Lisboa Norte, Avenida Professor Egas Moniz, 1649 Lisboa, PortugalDepartment of Cardiology, Hospital de Santa Maria, Centro Hospitalar de Lisboa Norte, Avenida Professor Egas Moniz, 1649 Lisboa, PortugalDepartment of Internal Medicine, Hospital Geral de Santo António, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099 Porto, PortugalIntroduction. 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, P=0.015). 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.http://dx.doi.org/10.1155/2013/489574 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Rui Baptista José Meireles Ana Agapito Graça Castro António Marinho da Silva Teresa Shiang Fabienne Gonçalves Susana Robalo-Martins António Nunes-Diogo Abílio Reis |
spellingShingle |
Rui Baptista José Meireles Ana Agapito Graça Castro António Marinho da Silva Teresa Shiang Fabienne Gonçalves Susana Robalo-Martins António Nunes-Diogo Abílio Reis Pulmonary Hypertension in Portugal: First Data from a Nationwide Registry BioMed Research International |
author_facet |
Rui Baptista José Meireles Ana Agapito Graça Castro António Marinho da Silva Teresa Shiang Fabienne Gonçalves Susana Robalo-Martins António Nunes-Diogo Abílio Reis |
author_sort |
Rui Baptista |
title |
Pulmonary Hypertension in Portugal: First Data from a Nationwide Registry |
title_short |
Pulmonary Hypertension in Portugal: First Data from a Nationwide Registry |
title_full |
Pulmonary Hypertension in Portugal: First Data from a Nationwide Registry |
title_fullStr |
Pulmonary Hypertension in Portugal: First Data from a Nationwide Registry |
title_full_unstemmed |
Pulmonary Hypertension in Portugal: First Data from a Nationwide Registry |
title_sort |
pulmonary hypertension in portugal: first data from a nationwide registry |
publisher |
Hindawi Limited |
series |
BioMed Research International |
issn |
2314-6133 2314-6141 |
publishDate |
2013-01-01 |
description |
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, P=0.015). 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. |
url |
http://dx.doi.org/10.1155/2013/489574 |
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