Ebstein's anomaly in persistent common atrioventricualr canal

Area of Science:

• Cardiology
• Pediatric Cardiology
• Congenital Heart Disease

Background:

• Persistent atrioventricular canal (PAVC) is a complex congenital heart defect.
• Ebstein anomaly is a rare congenital heart condition affecting the tricuspid valve.

Observation:

• This report details two pediatric patients presenting with early-onset cyanosis.
• Autopsy revealed concurrent incomplete PAVC and Ebstein anomaly of the tricuspid valve in both cases.
• Clinical investigations in one patient showed electrocardiogram findings consistent with PAVC and angiocardiogram demonstrating a "goose-neck" deformity.

Findings:

• The simultaneous occurrence of PAVC and Ebstein anomaly was confirmed post-mortem.
• Ebstein anomaly can significantly modify the clinical and hemodynamic characteristics of PAVC.
• Diagnostic imaging may reveal subtle signs of Ebstein anomaly in PAVC patients.

Implications:

• Consider Ebstein anomaly in atypical presentations of persistent atrioventricular canal.
• The presence of Ebstein anomaly poses challenges for surgical correction of PAVC.
• Accurate diagnosis of combined defects is crucial for appropriate patient management and surgical planning.