Managing a Critically Ill Patient With Pulmonary Arterial Hypertension

Management of critically ill patients with pulmonary hypertension presents significant clinical challenges. Regardless of the underlying etiology or chronicity of pulmonary hypertension, superimposed acute illness can precipitate decompensated right ventricular failure (RVF) and death. Hospitalization, particularly in the ICU, is associated with high mortality, with RVF serving as the direct or indirect cause in most cases. In this article, we will focus on the management of acute decompensated RVF in patients with known pulmonary arterial hypertension (PAH). ICU management of the patient with PAH with RVF centers on the recognition and treatment of potentially reversible precipitants for decompensation and supportive strategies to optimize right ventricular (RV) function. Key goals include maintaining adequate oxygenation and tissue perfusion, correcting electrolyte and other metabolic abnormalities, optimizing fluid balance and RV preload, enhancing RV contractility, and reducing RV afterload. Continuous monitoring of cardiac function is essential, as is identifying and treating potential complications (eg, arrhythmias, acute kidney injury). When RVF persists despite maximal medical therapy, extracorporeal life support may be considered as a bridge to recovery or transplantation. In patients with advanced disease, multidisciplinary discussions aligned with patient and family preferences should guide the goals of care. Integration of palliative care specialists to manage symptoms and support caregivers remains a critical component of comprehensive ICU management for patients with PAH.