Anomalía de Ebstein en la edad pediátrica: algoritmos de manejo y cirugía de reconstrucción del cono de Da Silva

Abstract

ObjectivesThe objective of this study is to report our casuistry in the Ebstein's anomaly surgery in the last 10 years, to analyze the considerations for surgical decision making in the neonate and present our results in da Silva's “cone” tricuspid reconstruction. MethodsRetrospective observational study that includes 11 patients diagnosed with Ebstein's anomaly operated on at our center from January 2011 to August 2021. Two neonates underwent Starnes procedure and 9 (beyond the neonatal period) Da Silva's “cone” surgery. ResultsThe 2 neonates in follow-up are in Fontan's situation and in functional class i (New York Heart Association). The mean age of patients operated beyond the neonatal period was 9.1±6.0 years. There was no hospital mortality or at follow-up. As postoperative complications, one patient was placed on ECMO for severe right ventricular dysfunction. There was no AV block. The mean follow-up was 5.3 years. Follow-up transthoracic echocardiogram demonstrated a significant reduction in tricuspid regurgitation from 3.7±0.6 to 1.1±0.9 (P<.0001), and the New York Heart Association class improved from 2.3±0.7 to 1.2±0.4 (P<.001). There has been no immediate or follow-up tricuspid valve replacement. ConclusionsThe “Cone” procedure can be performed safely with low morbidity and mortality. In the medium-term follow-up, the echocardiogram shows a significant reduction in the degree of tricuspid regurgitation, the patients have improved their functional class and none have required tricuspid valve replacement.