Congenitally complete heart block. Developmental aspects.

Abstract

Three cases of congenitally complete heart block are described of hearts in which other minor congenital malformations were not in themselves severe enough to disrupt the atrioventricular conduction system. The cases fitted well into the categorization of complete heart block suggested by Lev. Two exhibited lack of communication between the atrial and conducting tissues, the other had discontinuity of the penetrating atrioventricular bundle. In an attempt to explain why this discontinuity between different segments of the conducting tissues occurs, we re-examined several series of graded human embryos. This investigation suggested that the anulus fibrosus in the normal heart is derived from sulcus tissue of the atrioventricular junction, the endocardial atrioventricular cushions playing a minor role in the separation of atria from ventricles. The relationships between the sulcus tissues and the different components of the atrioventricular junctional area are discussed in terms of an explanation both for the existence of different types of congenitally complete heart block and for persistence of Mahaim (nodo-ventricular and nodo-fascicular) fibers.