Neonatal Ebsteins Anomaly presenting with unique features: Rare case report

Abstract

Ebstein’s anomaly (EA) of the tricuspid valve is an uncommon malformation, representing an estimate 0.3% to 0.6% of all cases of congenital heart disease. The basic defect involves a deformed and displaced tricuspid valve, resulting in symptoms that vary with age of the patient and severity of the defect. Symptoms are referable to progressive heart failure and cyanosis resulting from severe tricuspid insufficiency. Once symptoms appear the only treatment for Ebstein’s anomaly is surgical intervention. Infants with symptomatic Ebstein’s anomaly have a poor prognosis, with an expected mortality rate of 50% to 75%. Previous attempts at palliative surgical treatment in this neonatal group have produced equally dismal results. Herein, we are reporting a very sick 3 day old neonate afflicted with Ebsteins anomaly with double-outlet left ventricle (DOLV) associated with myriads of other significant congenital heart defects.