{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,29]],"date-time":"2025-12-29T18:49:21Z","timestamp":1767034161859,"version":"3.37.3"},"reference-count":34,"publisher":"Wiley","license":[{"start":{"date-parts":[[2013,1,1]],"date-time":"2013-01-01T00:00:00Z","timestamp":1356998400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/3.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["BioMed Research International"],"published-print":{"date-parts":[[2013]]},"abstract":"<jats:p><jats:italic>Introduction<\/jats:italic>. Pulmonary arterial hypertension (PAH) is a rare disease that must be managed in specialized centers; therefore, the availability of epidemiological national data is critical.<jats:italic>Methods<\/jats:italic>. 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).<jats:italic>Results<\/jats:italic>. 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 \u00b1 16.4 years. Idiopathic PAH was the most common etiology (37%). At presentation, PAH patients had elevated right atrial pressure (RAP) (7.7 \u00b1 5.9\u2009mmHg) and mean pulmonary vascular resistance (11.4 \u00b1 6.5 Wood units), with a low cardiac index (2.7 \u00b1 1.1\u2009L\u00b7min<jats:sup>\u22121<\/jats:sup>\u00b7m<jats:sup>\u22122<\/jats:sup>); 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 \u00b1 12.5 years) and had higher RAP (11.0 \u00b1 5.2\u2009mmHg,<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M1\"><mml:mi>P<\/mml:mi><mml:mo>=<\/mml:mo><mml:mn>0.015<\/mml:mn><\/mml:math>).<jats:italic>Conclusions<\/jats:italic>. 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.<\/jats:p>","DOI":"10.1155\/2013\/489574","type":"journal-article","created":{"date-parts":[[2013,10,21]],"date-time":"2013-10-21T18:01:28Z","timestamp":1382378488000},"page":"1-8","source":"Crossref","is-referenced-by-count":39,"title":["Pulmonary Hypertension in Portugal: First Data from a Nationwide Registry"],"prefix":"10.1155","volume":"2013","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7411-7039","authenticated-orcid":true,"given":"Rui","family":"Baptista","sequence":"first","affiliation":[{"name":"Department of Cardiology, Hospitais da Universidade de Coimbra, Centro Hospitalar e Universit\u00e1rio de Coimbra, Praceta Mota Pinto, 3000 Coimbra, Portugal"},{"name":"Institute for Biomedical Imaging and Life Sciences, Faculty of Medicine of University of Coimbra, Azinhaga de Santa Comba, 3000 Coimbra, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0243-1897","authenticated-orcid":true,"given":"Jos\u00e9","family":"Meireles","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Hospital Geral de Santo Ant\u00f3nio, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099 Porto, Portugal"}]},{"given":"Ana","family":"Agapito","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Hospital Santa Marta, Centro Hospitalar de Lisboa Central, Rua de Santa Marta, 1169 Lisboa, Portugal"}]},{"given":"Gra\u00e7a","family":"Castro","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Hospitais da Universidade de Coimbra, Centro Hospitalar e Universit\u00e1rio de Coimbra, Praceta Mota Pinto, 3000 Coimbra, Portugal"}]},{"given":"Ant\u00f3nio","family":"Marinho da Silva","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Hospitais da Universidade de Coimbra, Centro Hospitalar e Universit\u00e1rio de Coimbra, Praceta Mota Pinto, 3000 Coimbra, Portugal"}]},{"given":"Teresa","family":"Shiang","sequence":"additional","affiliation":[{"name":"Department of Pneumology, Hospital Santos Silva, Centro Hospitalar Gaia\/Espinho, Rua Concei\u00e7\u00e3o Fernandes, 4434 Vila Nova de Gaia, Portugal"}]},{"given":"Fabienne","family":"Gon\u00e7alves","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Hospital Geral de Santo Ant\u00f3nio, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099 Porto, Portugal"}]},{"given":"Susana","family":"Robalo-Martins","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Hospital de Santa Maria, Centro Hospitalar de Lisboa Norte, Avenida Professor Egas Moniz, 1649 Lisboa, Portugal"}]},{"given":"Ant\u00f3nio","family":"Nunes-Diogo","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Hospital de Santa Maria, Centro Hospitalar de Lisboa Norte, Avenida Professor Egas Moniz, 1649 Lisboa, Portugal"}]},{"given":"Ab\u00edlio","family":"Reis","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Hospital Geral de Santo Ant\u00f3nio, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099 Porto, Portugal"}]}],"member":"311","reference":[{"issue":"5","key":"1","doi-asserted-by":"crossref","first-page":"343","DOI":"10.7326\/0003-4819-115-5-343","volume":"115","year":"1991","journal-title":"Annals of Internal Medicine"},{"key":"2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra040291"},{"key":"3","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00007311"},{"key":"4","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.109.911818"},{"key":"5","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00006411"},{"key":"7","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2009.04.012"},{"key":"8","first-page":"S7","volume":"17","year":"2010","journal-title":"Revista Portuguesa de 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