Circulation, Vol 67, 626-631, Copyright © 1983 by American Heart Association
JE Deanfield, SY Ho, RH Anderson, WJ McKenna, SP Allwork and KA Hallidie-Smith
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.
ARTICLES
Late sudden death after repair of tetralogy of Fallot: a clinicopathologic study
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