Treatment Strategies for Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a progressive disease marked by vasoconstriction and vascular remodeling within pulmonary arteries leading to an increase in pulmonary vascular resistance, right heart failure, and death. Significant advances in understanding the molecular mechanisms and pathobiology of PAH have identified three key pathways involved in progression of this disease, specifically the endothelin, the prostacyclin, and the nitric oxide/cyclic guanosine monophosphate (cGMP) pathways. Echocardiogram is the best screening tool when clinical suscipicion of PAH exists to obtain an estimation of the pulmonary artery systolic pressure and to assess structure and function of the right ventricle. Right heart catheterization is necessary to diagnose PAH. There are currently eight PAH-specific therapies aimed at restoring the balance within the pulmonary vascular endothelium by augmenting the deficiency in prostacyclin, enhancing the nitric-oxide-mediated effects, and blocking the actions of endothelin. Active research continues in assessing the application of pulmonary vasodilators in patients with pulmonary hypertension due to left heart disease and in developing new drugs targeting novel mechanisms. Significant progress has been achieved in improving clinical outcome in PAH, yet there is a great need to develop more effective therapeutic strategies.