Heart failure and pulmonary hypertension.

When pulmonary hypertension (PH) and right ventricular dysfunction accompany heart failure, the impact on functional capacity and prognosis are ominous. Newer clinical strategies to preferentially lower pulmonary pressures and pulmonary vascular tone improve functional performance and symptoms of heart failure by targeting the nitric oxide signal transduction pathways, as with PDE5 inhibition. Additional studies are needed to determine if these therapies will impact long-term patient outcomes and elucidate the specific mechanisms whereby these treatments are effective. Furthermore, the recent finding that mutations in BMPR2 cause familial forms of pulmonary arterial hypertension and that BMPR2 expression is decreased in secondary forms of PH strongly implicate BMP signaling in the underlying pathophysiology of PH. Translation of emerging basic science insights in the vascular biology of PH and BMP signaling will provide novel therapeutic strategies for the spectrum of pulmonary hypertensive diseases.