Management of severe pulmonary arterial hypertension.

Despite advances in medical therapies, pulmonary arterial hypertension (PAH), continues to cause significant morbidity and mortality. Although, the right ventricle can adapt to an increase in afterload, progression of the pulmonary vasculopathy that characterizes PAH causes many patients to develop progressive right ventricular (RV) failure. Furthermore, acute RV decompensation may develop from disorders that lead to either an acute increase in cardiac demand or an increase in ventricular afterload including interruptions in medical therapy, arrhythmia, or pulmonary embolism. The poor reserve of the right ventricle, RV ischemia, and adverse RV influence on left ventricular filling may lead to a global reduction in oxygen delivery and multiorgan failure. The authors present an approach to patients with advanced PAH focusing on both medical and surgical strategies to improve RV function based upon current evidence and physiological principles.