Management of Ebstein's Anomaly and Pure Tricuspid Insufficiency in the Neonate

Because the pulmonary vascular resistance is very elevated at birth, severe tricuspid regurgitation is poorly tolerated and even life-threatening in the newborn. The etiology may be tricuspid valve papillary muscle rupture or the more ominous Ebstein's anomaly, with its associated dysfunctional right ventricle. After the diagnosis is established and the patient is supported with prostaglandin infusion and nitric oxide, definitive surgical management is undertaken with the expectation of excellent outcomes for isolated tricuspid valve regurgitation. For neonates with Ebstein's anomaly, therapy is tailored to the severity of the malformation and the degree of right ventricular outflow tract obstruction, assessed in the context of declining pulmonary vascular resistance. The surgical approach may involve ligation of a patent ductus arteriosus, placement of a systemic to pulmonary shunt, establishment of functional tricuspid atresia, or tricuspid valve repair. With the application of these various approaches, the outlook for neonatal Ebstein's anomaly has improved remarkably.