Rationale: Patients with pulmonary hypertension (PH) admitted to pediatric cardiac ICUs are at high risk of mortality. Objectives: To identify factors associated with mortality in cardiac critical care admissions with PH. Methods: We evaluated medical admissions with PH to Pediatric Cardiac Critical Care Consortium institutions over 5 years. PH was standardly defined in the clinical registry by diagnosis and/or receipt of intensive care-level pulmonary vasodilator therapy. Multivariable logistic regression identified independent associations with mortality. Measurements and Main Results: We analyzed 2,602 admissions; mortality was 10% versus 3.9% for all other medical admissions. Covariates most strongly associated with mortality included invasive ventilation (adjusted odds ratio, 44.8; 95% confidence interval, 6.2-323), noninvasive ventilation (19.7; 2.8-140), cardiopulmonary resuscitation (8.9; 5.6-14.1), and vasoactive infusions (4.8; 2.6-8.8). Patients receiving both invasive ventilation and vasoactive infusions on admission Days 1 and 2 had an observed mortality rate of 29.2% and 28.6%, respectively, compared with <5% for those not receiving either. Vasoactive infusions emerged as the dominant early risk factor for mortality, increasing the absolute risk of mortality on average by 6.4% when present on admission Day 2. Conclusions: Patients with PH admitted to pediatric cardiac critical care units have high mortality rates. Those receiving invasive ventilation and vasoactive infusions on Day 1 or Day 2 had an observed mortality rate that was more than fivefold greater than that of those who did not. These data highlight the illness severity of patients with PH in this setting and could help inform conversations with families regarding the prognosis.
Keywords: cardiac critical care; outcomes; pediatric cardiology; pulmonary vascular disease.