Key result
Surgery addresses a rare combination of Ebstein's anomaly, multiple VSDs, and severe pulmonary stenosis.
Case Report (n=1)
No
This case report describes the surgical management of a rare presentation of Ebstein's anomaly complicated by multiple VSDs and severe pulmonary stenosis in an adult.
Supports surgical feasibility in rare adult Ebstein's variant; extends sparse literature but leaves optimal strategies open.
Ebstein's anomaly is a rare and complexed heart defect that affects the tricuspid valve and is accountable for around 1% of congenital cardiac abnormalities. It is one of the most common congenital causes of tricuspid valve regurgitation. Ebstein's anomaly is often diagnosed prenatally due to its severe cardiomegaly. Some individuals with this anomaly do not experience any complications until adulthood and even then its mostly minor complaints like exercise intolerance. Atrial septal defect is most commonly (70-90%) associated with Ebstein's anomaly. However, ventricular septal defect (VSD) can be associated with 2-6% of the cases. This particular report presents a case of surgical intervention for a 20 years old female with Ebstein's anomaly that had multiple VSD's and a severe Pulmonary Stenosis (PS).
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Gupta et al. (2020) conducted a case report in Ebstein's anomaly with multiple VSDs and severe Pulmonary Stenosis (n=1). Surgical intervention was evaluated. Surgical intervention was performed on a 20-year-old female presenting with a rare combination of Ebstein's anomaly, multiple ventricular septal defects, and severe pulmonary stenosis.
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