Isolated congenitally complete heart block attributable to combined nodoventricular and intraventricular discontinuity

Intraventricular together with atrial-axis and nodoventricular discontinuity, in which various parts of the conduction system are replaced by fibrous or fatty tissue, constitute the three major pathological categories of isolated congenitally complete heart block. Intraventricular discontinuity is distinctly rare, with only two previous cases reported in the literature, one of which was associated with a familial history of heart block. The cardiac conduction systems of two cases of isolated congenitally complete heart block were serially sectioned and analyzed histopathologically. The findings were correlated with the clinical features, in particular, the family histories and maternal serum anti-Re antibodies. Both cases, a 9-day-old neonate and an 8-year-old schoolgirl, showed a combination of nodoventricular and intraventricular discontinuity with absence of the atrioventricular penetrating bundle, the entire right, and the proximal portion of the left bundle branch. The branching bundle was absent in the first case and replaced by fatty tissue in the second. In contrast to the commoner atrial-axis discontinuity in which the atrioventricular node itself is usually replaced by fibrous or fatty tissue with variable involvement distally, the sinus node, and in particular, the atrioventricular node were normal in both of our cases. There was no family history in either case, whereas tests for the maternal serum anti-Re antibody were positive in the first but negative in the second case. Intraventricular discontinuity as a cause of isolated congenitally complete heart block is very rare. In our cases, it coexisted with nodoventricular discontinuity. It can be sporadic, familial, or associated with positive maternal serum anti-Re antibodies. Copyright (C) 1998 by W.B. Saunders Company.