Chapter 31 - Neonates With Critical Congenital Heart Disease: Delivery Room Management and Stabilization Before Transfer to the Cardiac Intensive Care Unit

Abstract

Complex forms of congenital heart disease (CHD) often require early and or emergent medical, surgical, or catheterization–based interventions in the first days to weeks of postnatal life. CHD lesions with inadequate aortic or pulmonary outflow can usually be stabilized in the neonatal period with medical therapy, using prostaglandin E1 to maintain ductal patency, prior to intervention. More critical forms of CHD have an additional element of instability during perinatal transition and may need emergent neonatal intervention in the first hours after delivery. These include lesions such as obstructed total anomalous pulmonary venous return and hypoplastic left heart syndrome with restrictive atrial septum, which lack adequate pulmonary egress, d-transposition of the great arteries with restrictive atrial septum resulting in limited intracardiac mixing, and severe Ebstein anomaly and tetralogy of Fallot with absent pulmonary valve associated with airway anomaly, which compromises the caregivers’ ability to ventilate, and congenital complete heart block with inadequate cardiac output due to severe fetal bradycardia. These critical forms of CHD can be predicted with fetal echocardiography, which then allows for careful planning of maternal care to optimize the delivery and provide targeted postnatal care. Many cardiac centers are now using highly coordinated perinatal action plans that are lesion-specific to guide neonatal stabilization and, if appropriate, immediate transfer to the cardiac care team. Active perinatal strategies and delivery room protocols based on the anticipated postnatal acuity level, complexity, and need for emergent intervention hold the promise of improving outcomes for neonates with complex CHD. In the future, innovative fetal therapies may also alter outcomes of critical CHD and allow for improved transition from fetal to postnatal life.