pediatrics
Ebstein Anomaly
Treatment of Ebstein Anomaly
Medications Surgery Interventional catheterization Medications play a role in managing neonates who are severely cyanotic and have inadequate pulmonary blood flow. Maintaining ductal patency with prostaglandins allows time for the pulmonary vascular resistance to drop. The decrease in pulmonary artery pressure allows the compromised right ventricle to provide adequate pulmonary blood flow. Some neonates with very severe Ebstein anomaly and functional compromise of the right ventricle require more prolonged palliation with a Blalock–Thomas–Taussig (BTT) shunt or other complex surgical interventions. After the first several months of age, surgical repair of the tricuspid valve should be considered when any symptoms are present or there is evidence of progressive tricuspid valve dysfunction or enlargement of the right heart chambers. Surgical treatment of this anomaly has evolved over the years with developments such as the cone procedure ( Treatment reference ). This approach to repair of the tricuspid vale involves delamination and mobilization of the tricuspid valve leaflets, plicating the atrialized portion of the right ventricle and the annulus and reattaching newly created "leaflet cone" to the true tricuspid annulus. These advances have greatly enhanced the surgeon's ability to achieve an excellent functional repair of even the most deformed tricuspid valves. Any atrial defect is closed at the same time. Interventional catheterization techniques may be used to close an atrial communication when cyanosis is present, but the tricuspid valve dysfunction is only modest. 1.da Silva JP, Baumgratz JF, da Fonseca L, et al: The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results.J Thorac Cardiovasc Surg133(1):215–223, 2007. doi:10.1016/j.jtcvs.2006.09.018 