Circulation, Vol 90, 1310-1318, Copyright © 1994 by American Heart Association
A Matitiau, A Perez-Atayde, SP Sanders, T Sluysmans, IA Parness, PJ Spevak and SD Colan
BACKGROUND: For patients with acute dilated cardiomyopathy, definition of
prognosis and of clinical features predictive of outcome is particularly
important due to the availability of cardiac transplantation and other
innovative treatment strategies. METHODS AND RESULTS: We reviewed our
experience with 24 children under 2 years of age with dilated congestive
cardiomyopathy to determine outcome and potential predictive variables.
Clinical, serological, ECG, echocardiographic, hemodynamic, and
histological findings were analyzed. Idiopathic cardiomyopathy or
myocarditis constituted 29% of the patients presenting with congestive
heart failure without structural heart disease. Among these patients, 45%
recovered completely, 25% survived with persistent left ventricular
dysfunction, and 30% died. All except one of the deaths occurred during the
first 2 months after presentation. Poorer outcome and higher mortality were
associated with a more severely depressed left ventricular ejection
fraction and/or a more spherical left ventricular shape at presentation.
Histological evidence of myocardial inflammation was a favorable prognostic
indicator, whereas histological evidence of endocardial fibroelastosis was
associated with a poor outcome. During the recovery phase, diastolic volume
fell rapidly. Ventricular mass was elevated from the earliest observations
and fell more slowly, with persistent elevation of the mass-to-volume ratio
up to 2 years. Function and contractility improved over the first several
months in most patients who recovered, although in occasional patients
continued improvement was seen for as long as 2 years after presentation.
CONCLUSIONS: Histological and echocardiographic features can be used to
identify patients at particularly high risk for death. To have any impact
on outcome, decisions about cardiac transplantation must be reached
rapidly, since almost all deaths occurred within the first 2 months after
presentation. Recovery of function is often rapid, but continued
improvement may be seen for as long as 2 years.
ARTICLES
Infantile dilated cardiomyopathy. Relation of outcome to left ventricular mechanics, hemodynamics, and histology at the time of presentation
Department of Cardiology, Children's Hospital, Harvard Medical School, Boston, Mass. 02115.
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