New and future therapies in pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is defined as a mean pulmonary artery pressure greater then 25 mm Hg at rest and greater then 30 mm Hg with exercise in association with normal left heart filling pressures. However, many patients with PAH have much higher pulmonary artery pressures, which, untreated, can rapidly result in marked disability, right heart failure, and death. A myriad of recent therapeutic advances have revolutionized the treatment of PAH, significantly impacting both symptoms and survival. This article briefly discusses some of the medication classes currently approved to treat patients with PAH and reviews novel therapies in the context of recent clinical trials. Also examined here are the rationale and data available for the role of combination therapy in the treatment of PAH. The article closes with speculation on new directions in the therapeutic management of this life-threatening disease.