Circulation, Vol 61, 441-450, Copyright © 1980 by American Heart Association
HP Gutgesell, ME Speer and HS Rosenberg
A transient form of hypertrophic cardiomyopathy has been previously
described in infants of diabetic mothers (IDMs). The purpose of this study
was to determine the incidence, natural history and pathologic features of
this cardiomyopathy in symptomatic and asymptomatic IDMs. We studied 47
IDMs for evidence of cardiomyopathy. Among 24 symptomatic IDMs, five had
marked septal hypertrophy with echocardiographic features suggesting left
ventricular outflow obstruction and five had hypertrophy of the right
ventricular free wall. With the exception of mild septal hypertrophy, these
abnormalities resolved during the first 6 months of life, and
echocardiograms in the first-degree family members were normal. Of 23
asymptomatic IDMs, three had septal hypertrophy and two had right
ventricular free wall hypertrophy; none of the asymptomatic IDMs had
evidence of outflow obstruction. One symptomatic IDM died, and autopsy
revealed a hypertrophic septum that distorted both ventricular cavities.
Microscopic examination revealed hypertrophic fibers and occasional areas
of cellular disarray in the septum. Despite the clinical and pathologic
similarities of the cardiomyopathy in IDMs to the hypertropic
cardiomyopathies in older children and adults, its transient and
nonfamilial nature suggest that it is a separate disease. We speculate that
it is a manifestation of the generalized organomegaly in IDMs.
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Characterization of the cardiomyopathy in infants of diabetic mothers
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