Update on therapies for pulmonary hypertension

Pulmonary hypertension (PH) is often difficult to diagnose and many different disorders may result in elevated pulmonary arterial pressure requiring therapy. Left untreated, PH usually has a dismal prognosis culminating in right ventricular failure and death. Besides conservative therapeutic strategies such as anticoagulation and diuretics, the past decade has brought remarkable improvements in therapy for the major classification groups of PH (pulmonary arterial and chronic thromboembolic pulmonary hypertension), based on a better understanding of the underlying pathobiology. Selection of appropriate therapies for PH remains complex and requires familiarity with the disease process, evidence from clinical trials, complicated drug delivery systems, dosing regimens, side effects and complications. Despite these advances, none of the current therapeutic pathways is curative. This article discusses the currently available drug therapy for PH, considers the surgical option for some patients with chronic thromboembolic disease, and looks forward to possible new forms of therapy emerging from bench research.