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Retrospective analysis was performed to determine the likely cause of death in six patients who died suddenly 1-9 years after repair of tetralogy of Fallot. The integrity of the atrioventricular conduction system and myocardium was examined in three hearts at necropsy. Two of three patients who complained of palpitation or syncope had frequent premature ventricular complexes on routine ECGs. All the patients had complete right bundle branch block and one had left-axis deviation after repair. Postoperative cardiac catheterization (four patients) showed no residual ventricular septal defects, but right ventricular pressure was elevated in all. At necropsy, the atrioventricular conduction tissue was related to the margin of perimembranous ventricular septal defect in two patients, but was well posterior in one patient with an infundibular muscular defect. Histologic examination showed that in all cases the atrioventricular node, atrioventricular bundle and left bundle branch were undamaged. There was, however, extensive fibrosis of the right ventricular myocardium in the ventriculotomy site (three patients), septum (one patient) and outflow tract (one patient). These clinical and necropsy findings suggest that ventricular arrhythmia rather than atrioventricular block was responsible for sudden death in these patients after repair of tetralogy of Fallot.

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Our findings indicate that the defect requires initial formation of the walls of the coronary sinus, but with subsequent dissolution of the wall adjacent to the left atrium. This produces a communication between the atriums through the mouth of the sinus.

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