Pulmonary arterial hypertension – Experience of Centro Hospitalar de Vila Nova de Gaia

Abstract

IntroductionPulmonary arterial hypertension (PAH) is a serious and progressive disease, potentially fatal in a few years. Until the 1990s the only effective treatment for this disease was pulmonary/cardiopulmonary transplant. Over the last few years several drugs have emerged that have modified the course of the disease. AimsTo characterise patients with PAH followed at Centro Hospitalar de V.N.Gaia between 2000 and 2006. To raise awareness of this entity. Material and methodsRetrospective analysis of all patients with PAH or chronic thromboembolic pulmonary hypertension (CTEPH) followed at our Hospital. ResultsIn this period 11 patients were observed, 7 women and 4 men, with a mean age of 43 y. Five patients had idiopathic PAH, three had CTEPH, two had PAH associated with interauricular communication and one had PAH associated with CREST Syndrome.At the time of diagnosis most patients were in NYHA//WHO Functional Class III/IV, mean systolic pulmonary artery pressure was 98±32mmHg and mean distance walked in the 6-minute walk test was 401m. Initial treatment: Three patients had inhaled iloprost, two subcutaneous treprostinil, three oral bosentan, one oral sildenafil and two patients underwent surgery. Mean follow-up time was 28.1 months. Five patients recovered, three are stable, one worsened and two died. ConclusionsPAH is a serious illness which requires a multidisciplinary approach with the need for complex and expensive therapies. Diagnosis in initial stages is essential for an effective treatment.