Managing an acutely ill patient with pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a progressive disease characterized by a vasculopathy that results in sustained elevation of pulmonary artery pressures, which ultimately leads to right ventricular failure (RVF) and death. Several advances have been made in the treatment of PAH, but it remains a major cause of morbidity and mortality. Managing an acutely ill patient with PAH is especially challenging. The mechanisms of RVF are incompletely understood; these patients do not tolerate fluid shifts well and there are no controlled trials to assess superiority of a certain approach. This article outlines an approach based on current understanding of RVF in PAH and recommends an approach based on the pathophysiology, current evidence and experience.