Severe Ebstein's anomaly can benefit from a small ventricular septal defect: two cases

Area of Science:

• Cardiology
• Pediatric Cardiology
• Congenital Heart Disease

Background:

• Ebstein's anomaly is a rare congenital heart defect with high neonatal mortality.
• Associated cardiac lesions are infrequent in Ebstein's anomaly.
• The neonatal period represents the highest risk for mortality in affected infants.

Observation:

• This report details two cases of severe Ebstein's anomaly co-occurring with a ventricular septal defect.
• The presence of a ventricular septal defect was noted in both severe Ebstein's anomaly cases.
• Neonatal survival was observed in these patients despite the severity of Ebstein's anomaly.

Findings:

• The co-occurrence of Ebstein's anomaly and ventricular septal defect may promote adequate pulmonary blood flow in neonates.
• This physiological adaptation potentially allows survival through the critical neonatal period.
• A small ventricular septal defect might be a protective factor in severe Ebstein's anomaly.

Implications:

• The findings suggest that a ventricular septal defect could be beneficial in severe Ebstein's anomaly.
• This association may obviate the need for high-risk early infancy surgical intervention.
• Further research into this rare association could refine management strategies for Ebstein's anomaly.