RESUMO
Background: Pulmonary arterial hypertension is an important cause ofcomplica-tions amongpatients with connective tissue diseases. Aim: To describe the clinical and echocardiographic characteristics ofpatients with pulmonary hypertension associated with connective tissue diseases. Material and Methods: Retrospective, observational and descriptive study. We analyzed 35 patients with pulmonary hypertension associated with connective tissue diseases. All patients were evaluated and diagnosed by at least one medical specialist in rheumatology. Pulmonary arterial hypertension was defined as a pulmonary artery systolic pressure ≥ 40 mmHg by echocardiography. The group was divided as not severe when pressures ranged from 40 to 64 mmHg and severe, when pressures were ≥ 65 mmHg. Results: The most common connective tissue disease associated with pulmonary arterial hypertension was diffuse scleroderma in 46% of cases. Eighty nine percent of patients were female. Time of evolution of the pulmonary hypertension was 18.8 ± 21.8 months. The distance walked in the six minute walk test was < 400 m both in patients with and without severe pulmonary hypertension. Fifty one percent ofpatients had pulmonary restriction. No differences in gas exchange parameters were observed between groups. Comparing echocardio-graphic findings in patients with and without severe hypertension, the former had a higher frequency ofright ventricular dilatation (85.7 and 52.3% respectively, p = 0.04), right ventricular hypertrophy (42.8 and 0% respectively, p = 0.02) and right ventricular hypokinesia (71.4 and 9.5% respectively p = < 0.01). Conclusions: Patients with severe pulmonary arterial hypertension associated to connective tissue diseases have more commonly dilated, hypertrophic and hypokinetic right ventricles.