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[Complete atrioventricular block and sudden death in isolated corrected transposition]
Giornale Italiano Di Cardiologia
|August 1, 1986
Summary
Corrected transposition of the great arteries (cTGA) carries a high risk of atrioventricular (AV) conduction disturbances. This case highlights sclero-atrophy of the AV bundle as a cause of complete AV block and ventricular arrhythmias in isolated cTGA.
Area of Science:
- Cardiology
- Cardiac Electrophysiology
- Congenital Heart Disease
Background:
- Corrected transposition of the great arteries (cTGA) is a complex congenital heart defect.
- Patients with cTGA have an increased risk of atrioventricular (AV) conduction disturbances.
- The anatomical malposition of the specialized AV junction in cTGA may predispose to conduction abnormalities.
Observation:
- A 54-year-old female with a history of "idiopathic" complete AV block experienced sudden cardiac death.
- Autopsy revealed isolated cTGA without other cardiac anomalies.
- Electrocardiogram (ECG) showed ventricular fibrillation preceded by ventricular tachycardia.
Findings:
- Histological examination of the conduction system demonstrated an anteriorly positioned AV node.
- The bundle of His had a prolonged subendocardial course within the pulmonary outflow tract.
- The pathological basis for the complete AV block was identified as sclero-atrophy of the common AV bundle, similar to acquired AV block.
Implications:
- This case underscores the significant risk of conduction system disease in isolated cTGA.
- The findings suggest a potential link between AV block and the development of life-threatening ventricular arrhythmias in cTGA.
- Understanding these mechanisms is crucial for risk stratification and management of patients with cTGA.