[A 62-year-old survivor with Ebstein's anomaly without right ventricular failure]

Area of Science:

• Cardiology
• Electrophysiology
• Congenital Heart Disease

Background:

• Ebstein's anomaly is a rare congenital heart defect affecting the tricuspid valve.
• Wolff-Parkinson-White (WPW) syndrome is a cardiac condition characterized by an extra electrical pathway.
• Co-occurrence of Ebstein's anomaly and WPW syndrome presents unique diagnostic and management challenges.

Observation:

• A 62-year-old woman presented with concussion, later developing syncope and a heart murmur.
• Electrocardiogram revealed Wolff-Parkinson-White (WPW) configuration and supraventricular tachycardia.
• Echocardiogram showed tricuspid valve abnormalities, including septal leaflet displacement and mild regurgitation.
• Intracardiac electrocardiogram during cardiac catheterization confirmed an atrialized right ventricle.

Findings:

• The patient was diagnosed with Ebstein's anomaly in conjunction with WPW syndrome.
• This represents an exceptionally rare presentation of Ebstein's anomaly surviving into the sixth decade without heart failure.
• The patient's survival without heart failure is attributed to a milder form of the anomaly and absence of other congenital heart defects.

Implications:

• This case underscores the variable clinical spectrum of Ebstein's anomaly.
• It highlights the possibility of prolonged survival into adulthood for select patients with Ebstein's anomaly and WPW syndrome.
• The findings suggest that milder forms of Ebstein's anomaly may allow for delayed onset of right ventricular failure, even into older age.