Abstract
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.
- © 2012 ERS