CICU / Post-op CHD Care

Congenital diaphragmatic hernia (CDH) is a field defect causing bilateral pulmonary hypoplasia and altered pulmonary vascular resistance, not a surgical emergency but a physiologic one requiring birth at ECMO-capable centers.[e303-c2,e303-c3,e303-c4,e303-c7] Survival to adolescence is 80–85%. Respiratory management prioritizes spontaneous breathing with minimal sedation; preductal saturations of 90% are adequate given fetal hemoglobin.[e303-c14,e303-c17] Nitric oxide offers no benefit; oxygen is the primary therapy.[e303-c19,e303-c20] ECMO candidacy (OI >40 for ≥4 hours) requires reversible disease; VA bypass is preferred over VV given frequent cardiac dysfunction and mediastinal shift.[e303-c15,e303-c22,e303-c24] The foregut dysmotility—not reflux—causes esophageal and gastric ectasia; Nissen fundoplication is contraindicated.[e303-c28,e303-c30,e303-c32] Long-term sequelae include Barrett's esophagitis, neurodevelopmental disorders (ADHD, autism), asymmetric chest growth, and thoracolumbar scoliosis requiring multidisciplinary follow-up.[e303-c34,e303-c35,e303-c36,e303-c37] Pulmonary hypertension in CDH persists beyond 3 weeks in 50% of patients; endothelin upregulation predicts outcome, and chronic prostacyclin therapy (Remodulin) yields 50% survival.[e10205-c2,e10205-c5,e10205-c6] Combined pressure-flow forces drive endothelial dysfunction and vascular remodeling more than flow alone.[e10205-c21,e10205-c22] Post-cardiac surgery acute kidney injury requiring dialysis occurs in 1% of infants, with 1-year survival of 46% versus 94% without dialysis; high-volume centers achieve threefold better hospital survival.[e12114-c1,e12114-c2,e12114-c3,e12114-c5]