Circulation, Vol 51, 743-748, Copyright © 1975 by American Heart Association
TN James, RJ Marilley Jr and HJ Marriott
A seventeen-year-old girl in apparent good health was found dead in bed one
morning. Two clinical features of note were multiple premature beats
present in her electrocardiograms for several years, and a tall thin
habitus without stigmata of Marfan's syndrome. At necropsy examination the
heart was grossly normal. At its margin abutting the central fibrous body
the atrioventricular (A-V) node exhibited numerous fronds and outcroppings,
some forming loop connections from one part of A-V node to another. Groups
of A-V nodal cells detached from the A-V node were also found connected to
the crest of the interventricular septum. There were large connections
directly from the His bundle to the crest of the interventricular septum
(Mahaim fibers). The A-V node artery was moderately narrowed but patent.
The possible significance of these findings in the pathogenesis of
re-entrant arrhythmias or ectopic automaticity is discussed.
ARTICLES
De subitaneis mortibus. XI. Young girl with palpitations
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