Abstract
We report an innovative treatment strategy for fetal Ebstein's anomaly with a circular shunt. We used transplacental non-steroidal anti-inflammatory drugs, at the 29th gestational week, to constrict the ductus arteriosus avoiding fetal demise. We addressed the critical neonate with an urgent Starnes procedure. Finally, instead of following the usual single-ventricle palliation pathway after the Starnes procedure, we achieved successful two-ventricle repair with the cone technique at 5 month old.
Highlights
Brief ReportCite this article: Lopes LM, Bezerra RF, da Silva JP, and da Fonseca da Silva L (2021) Critical Ebstein’s anomaly with circular shunt: from successful fetal therapy with nonsteroidal anti-inflammatory drugs to biventricular repair using Da Silva cone technique
Ebstein’s anomaly diagnosed in fetal life is associated with high perinatal mortality.[1,2] A recent multicenter study showed that the risk of fetal demise or neonatal death was approximately 45%.3 Pulmonary insufficiency with circular shunt was the most important predictive risk factor for perinatal mortality
The circular shunt is prompted by the combination of pulmonary insufficiency and tricuspid regurgitation, causing continuous retrograde ductal flow and intracardiac recirculation, which results in low tissue perfusion and a life-threatening condition.[4]
Summary
Cite this article: Lopes LM, Bezerra RF, da Silva JP, and da Fonseca da Silva L (2021) Critical Ebstein’s anomaly with circular shunt: from successful fetal therapy with nonsteroidal anti-inflammatory drugs to biventricular repair using Da Silva cone technique. Received: 18 November 2020 Revised: 31 December 2020 Accepted: 1 January 2021 First published online: 8 March 2021. Critical Ebstein’s anomaly with circular shunt: from successful fetal therapy with non-steroidal anti-inflammatory drugs to biventricular repair using Da Silva cone technique. Bezerra[3], Jose Pedro da Silva[4] and Luciana da Fonseca da Silva[4]
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