Management of Pediatric Pulmonary Hypertension in the Intensive Care Unit

Pulmonary hypertension (PH) in children and adolescents is a multifactorial condition affecting the pulmonary vasculature that can lead to life-threatening complications, especially during acute decompensations. There is sudden/progressive right heart failure (RHF) with circulatory insufficiency and multiorgan hypoxemia during PH crises. Early diagnosis, individualized approach, and hemodynamic assessment are vital for effective management. Echocardiography, followed by radiodiagnostic imaging and cardiac catheterisation, are important diagnostic tools. A multifaceted approach prioritises lowering pulmonary vascular resistance, improving right ventricular function, and optimising cardiac output to control PH crises in the intensive care setting effectively. This review outlines the pathophysiology, clinical presentation, diagnosis, and management of acute PH in the pediatric intensive care unit (ICU). Therapeutic options range from cardinal ICU measures and targeted pharmacotherapy to advanced interventions such as extracorporeal life support and transplantation. This review highlights current evidence, enumerates recent advances in management, identifies gaps in knowledge, and underscores the need for further research to improve outcomes in pediatric acute PH.