[Disproportionate pulmonary arterial hypertension and lung respiratory diseases: distinctive clinical, hemodynamic and prognosis of patients versus primary pulmonary arterial hypertension].

OBJECTIVES: To characterize and compare patients with disproportionate PH versus patients with primary pulmonary arterial hypertension (PAH).

METHODS: All patients referred to our cardiology unit for echocardiography from November 2006 to May 2008 and who have been followed by our pneumologist were screened for severe PH (i.e mean arterial pulmonary pressure>35-40 mmHg at rest). Patients were excluded if a factor that could influence pulmonary hemodynamics was present. We investigated these patients by pulmonary function tests, echocardiography and right heart catheterisation.

RESULTS: We reported 16 cases of severe PH in stable patients (n=8, chronic obstructive pulmonary disease-emphysema) and 13 patients with PAH. Our findings suggest that the patients with disproportionate PH had right heart dysfunction similar to that observed in PAH. But their outcomes were more severe. It seemed that specific vasodilatator therapy was not efficient.