Key points Pulmonary arterial hypertension is still a chronic and incurable disease The most common mutation found in familial pulmonary arterial hypertension is a defect in the bone morphogenetic protein receptor 2 gene Usually the final stage of the disease is right heart failure Educational aims To provide definitions and diagnostic methods for pulmonary hypertension To provide a general classification of pulmonary hypertension To provide an overview of the currently available treatments To provide a future vision of next-generation treatments