[Clinical, functional and hemodynamic features of patients with pulmonary arterial hypertension]

Pulmonary Arterial Hypertension is a rare, progressive and devastating disease with severe consequences in quality of life and survival.

A clinical, functional and hemodynamic assessment of patients with pulmonary arterial hypertension and categorization according to severity.

Prospective registry of patients with arterial pulmonary hypertension, hemodynamically defined. Clinical evaluation was performed using World Health Organization functional score (I to IV) and Borg dyspnea scale. Six minute walking test, echocardiography and right heart catheterization were used for functional and hemodynamic assessment. Intravenous Adenosine was used to assess vascular reactivity during the hemodynamic evaluation.

Twenty nine patients were included (25 women, age range 16-72 years). Pulmonary hypertension was idiopathic in 11, associated to connective tissue disease in seven, associated to congenital heart disease in nine and associated to chronic thromboembolism in two. The mean lapse of symptoms before assessment was 2.9 years and 100% had dyspnea (Borg 5.1). Functional class I, II, III and IV was observed in 0, 5, 21 and 3 patients respectively. Six minutes walking test was 378+/-113 m. Mean pulmonary pressure was 59.4+/-12.2 mmHg, cardiac index was 2.57+/-0.88 and pulmonary vascular resistance index: 1798.4+/-855 (dyne.sec)/cm5. Nine patients had a mean pulmonary arterial pressure >55 mmHg and a cardiac index <2.1, considered as bad prognosis criteria. Adenosine test was positive in 17%.

This group of patients with Pulmonary Arterial Hypertension was mainly conformed by young females, with a moderate to severe disease.