[III-PSY4-1] Management of the fetus and neonate with Ebstein anomaly
Ebstein anomaly of the tricuspid valve and tricuspid valve dysplasia in the fetus and neonate some of the most difficult congenital heart defects to manage.In the fetus, the anatomic and physiologic progression can be severe resulting in fetal demise or premature birth. A child born prematurely with significant tricuspid valve disease can be very challenging to manage. Severe tricuspid regurgitation in the fetus can result in anatomic and physiologic aberrations, most importantly reversal of flow in the ductus arteriosus and more of a pulmonary regurgitation. Pulmonary regurgitation results from low right ventricular pressure with the right ventricle and able to eject antegrade across the pulmonary valve. This results in a circular shunt with poor systemic perfusion, acidosis and either fetal demise or premature birth. In some cases, NSAIDs can be used to promote ductal constriction to improve fetal physiology. This in turn may prolong the pregnancy and improve outcomes with a more mature newborn.In the neonatal period, decision making regarding the best management pathway is critical. In some patients, good intensive care management with the use of nitric oxide and allowing the ductus arteriosus to close may improve the physiology. If the child is critically ill a Starnes procedure is the best way to manage patients to get them out of the neonatal period. Later on tricuspid valve repair can be performed with a 1-1/2 ventricle repair or even a biventricular repair. Many of these patients also have a thin-walled right ventricular myopathy in addition to the Ebstein anomaly. We continue to learn and develop creative strategies to manage this complex set of patients.