Introduction: Pulmonary arterial hypertension (PAH) is a rare condition characterised by progressively elevated pulmonary arterial resistance leading to right heart failure.
State of the art: A recent classification distinguishes idiopathic PAH, familial PAH and PAH secondary to other conditions (connective tissue disease, congenital heart disease, portal hypertension, human immunodeficiency virus infection or appetite suppressant exposure). Echocardiography is the initial investigation of choice for non-invasive detection of PAH but measurement of pulmonary pressures and cardiac output during right-heart catheterization are necessary to confirm the diagnosis of PAH. Conventional treatment includes non-specific drugs (warfarin, diuretics, oxygen). Intravenous epoprostenol is the first-line treatment for the most severely affected patients. In less severe cases, the first-line treatment may include bosentan or a prostacyclin analogue.
Perspectives and conclusions: Recent advances in the management of PAH have markedly improved prognosis. The avai-lability of novel specific drugs including type 5 phosphodiesterase inhibitors offers novel therapeutic perspectives but their exact role in the treatment of PAH is still uncertain. The evolution of therapy from vasodilators to antiproliferative agents reflects the advancement in our understanding of the mechanisms mediating pulmonary arterial hypertension.