Improving survival in pulmonary arterial hypertension.

M ore than 50 yrs ago, DRESDALE et al. [1] reported a series of 39 patients with unexplained pulmonary hypertension and coined the term ‘‘primary pulmonary hypertension’’ to describe the condition, a term that has been revised to ‘‘idiopathic pulmonary arterial hypertension’’ in the most recent classification [2]. Idiopathic pulmonary arterial hypertension is a disease of the small pulmonary arteries, which leads to a progressive increase in pulmonary vascular resistance, ultimately causing right ventricular failure and death [3, 4]. Despite major advances in the understanding of the pathobiology of pulmonary arterial hypertension, none of the current therapeutic approaches achieves a cure for this devastating condition [5, 6].