Severe Pulmonary Hypertension in Critically Ill Children: A 10-Year Single-Center Case Series and Practical...

Severe pulmonary hypertension (PH) in children may decompensate rapidly during intercurrent illness and is associated with substantial morbidity and mortality in the pediatric intensive care unit (PICU). We describe the clinical presentation, intensive care management, and outcomes of four children with severe PH admitted to a tertiary PICU between January 2014 and December 2024. Three patients were infants, and one was an 11-year-old girl with a history of extreme prematurity and bronchopulmonary dysplasia. All four presented with acute respiratory distress, hypoxemia, and cardiovascular compromise following a recent respiratory tract infection. PH was documented predominantly by echocardiography, with findings including elevated estimated pulmonary pressures, right ventricular (RV) pressure overload, interventricular septal flattening, and/or RV dysfunction. Right heart catheterization was performed in one patient. All patients received supplemental oxygen, diuretics, and sildenafil. Two required invasive mechanical ventilation and vasoactive support, while one patient received additional prostanoid and endothelin receptor antagonist therapy. The median PICU length of stay was 15 days (range: 10-24 days). Two patients survived to hospital discharge, whereas two died following refractory pulmonary hypertensive crises. These cases highlight respiratory infection as an important potential precipitant of acute decompensation in children with severe PH and illustrate the challenges of supporting the failing RV in the PICU. Although the small sample size and retrospective design preclude conclusions regarding treatment efficacy or prognostic factors, the cases emphasize several practical management priorities: early recognition of deterioration, prompt correction of factors that increase pulmonary vascular resistance, preservation of RV function and systemic perfusion, cautious escalation of respiratory support, and early multidisciplinary involvement. Based on these principles and the published pediatric PH literature, we propose a practical, physiology-based management algorithm for severe PH and pulmonary hypertensive crisis in the PICU.