Circulation, Vol 51, 379-388, Copyright © 1975 by American Heart Association
TN James, RC McKone and AS Hudspeth
Complete heart block was found shortly after birth in a brother and sister
(not twins). Both were treated by electronic pacing because of symptoms
attributable to inadequate cardiac output and electrical instability of
heart. The boy has done well with his artificial pacemaker and is now six
years old. His sister died of complications due in part to the large size
of her pacemaker and small size of her body. At necropsy special studies of
her heart included the centers for normal impulse formation and concuction.
The primary abnormalities were at the junction of atrial septum with
atrioventricular (A-V) node, and at the origin of the two bundle branches
from the His bundle. The A-V node was isolated by collagen at all its
margins except its junction with the His bundle. The proximal His bundle
was essentially normal, but from that point on through the initial protions
of both the left and right bundle branches there was extensive caseous
degeneration which interrupted any possible conduction. These findings are
discussed in relation to fetal and postnatal development of the human A-V
node, and the His bundle and its branches; and in the context of a recently
observed mathematical relationship between sinus rate and two forms of
experimentally produced A-V junctional escape rhythms.
ARTICLES
De subitaneis mortibus. X. Familial congential heart block
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