[Idiopathic pulmonary arterial hypertension revealed by right ventricular dysfunction].

Abstract

Idiopathic pulmonary arterial hypertension (iPAH) is a rare, rapidly progressive disease associated with high morbidity and mortality. It is characterized by endothelial dysfunction within the pulmonary vascular bed and gradually leads to an increase in the pulmonary vascular resistances. Its non-specific symptomatology delays the diagnosis and brings the most severe forms to right ventricular failure. Management is based on haemodynamic assessment and specific vasodilator treatment. In iPAH, the vasoreactivity test during right heart catheterization is critical in order to identify a potential benefit of calcium channel blockers therapy leading to significant improvement of pulmonary haemodynamics.