Abstract
Pulmonary Arterial Hypertension (PAH) is a progressive vascular disease characterized by elevated Pulmonary Vascular Resistance (PVR) leading to Right Ventricular (RV) dysfunction and, ultimately, Right Heart Failure (RHF). Acute decompensation of PAH presents a life-threatening consequence marked by sudden worsening of clinical signs of right heart failure, systemic circulatory insufficiency, and multi-system organ failure. Clinicians are encountering more and more patients with PAH and RHF in the critical care units. These patients require admission and management in a critical care unit until they can be stabilized. The pathogenesis involves an imbalance between RV afterload and its adaptation capacity, ultimately resulting in RV dilation and failure. While the causes of acute decompensation remain subtle in many cases, infections, drug noncompliance, and pulmonary embolism are common culprits. Early identification of signs and symptoms of acute decompensation of RV failure, determination of possible etiology, and timely initiation of optimal treatment approaches are pivotal in avoiding detrimental outcomes. Optimization of pre-load and use of pulmonary vasodilators and inotropic agents are cornerstones of management. In refractory cases, mechanical circulatory support such as Extracorporeal Membrane Oxygenation (ECMO) or Right Ventricular Assist Devices (RVADs) may be necessary. Balloon Atrial Septostomy (BAS) serves as a bridge to definitive therapy, offering decompression of the right atrium and right ventricle. The prognosis of acute decompensated RV failure in PAH patients remains poor, highlighting the critical need for early diagnosis and intervention to improve outcomes. Currently, there are no strict standard guidelines to manage acute decompensated RV failure in PAH patients. We aim to revisit current evidence and practice trends in PAH and its acute decompensation.