TY - JOUR T1 - Pulmonary hypertension in Portugal: First data from a nationwide registry JF - European Respiratory Journal JO - Eur Respir J VL - 40 IS - Suppl 56 SP - 366 AU - Rui Baptista AU - José Meireles AU - Ana Agapito AU - Graça Castro AU - António Marinho da Silva AU - Teresa Shiang AU - Ana Oliveira AU - Daniela Ferreira AU - Fabienne Gonçalves AU - Susana Robalo-Martins AU - António Nunes Diogo AU - Abilio Reis Y1 - 2012/09/01 UR - http://erj.ersjournals.com/content/40/Suppl_56/366.abstract N2 - Pulmonary arterial hypertension (PAH) is a rare disease that must be managed in specialized centers integrated in a national network. Availability of epidemiological national data is critical for planning and regulation of healthcare in this field.We conducted a prospective, observational and multicenter registry in 5 portuguese centers. Adults with PAH and chronic thromboembolic PH (CTEPH) confirmed by right heart cath (RHC) were included.79 patients were enrolled; 46 (58.2%) classified as PAH and 33 (41.8%) as CTEPH. PAH patients had a mean age of 43.4±16.4 years and the f/m ratio 1.9:1. Idiopathic PAH was present in 17 (37%) patients, followed by connective tissue disease (n=12, 26%), congenital heart disease (n=10, 22%), portopulmonary (n=5, 11%), heritable (n=1, 2%) and other etiologies (n=1, 2%). At baseline most patients were WHO class III or IV (71%). Baseline RHC: elevated RAP (7.7±5.9 mmHg), mPAP (50.6±17.9 mmHg) and mean PVR (11.4±6.5 Wood U), with a low CI (2.7±1.1 L.min-1.m-2).At baseline patients were medicated with conventional therapies; at follow-up, most were on single (50%), double (28%) or triple (9%) combination of specific therapy. 1-year survival was 93.5%.CTEPH patients were older (60.0±12.5 years) and had higher RAP (11.0±5.2 mmHg, p = 0.015), but 1-year survival (93.9%) was similar to PAH patients. Five CTEPH patients underwent pulmonary endarterectomy.We estimated an annual incidence of 1.5 and 1.1 per million in PAH and CTEPH, respectively.Our report describes nationwide data on the diagnosis, management and clinical course of groups 1 and 4 PH patients. Clinical presentation, hemodynamics and survival are comparable with those reported on other national registries. ER -