Heart Diseases Clinical Trial
To determine the familial occurrence and pathogenesis of idiopathic dilated cardiomyopathy.
BACKGROUND:
In 1987 idiopathic dilated cardiomyopathy was a disorder of unknown cause that directly
affected one or both cardiac ventricles in a diffuse or multifactorial fashion, and that
produced heart failure, at least in some patients. Although a viral etiology had been
proposed, dilated cardiomyopathy could be associated with numerous genetic and non-genetic
diseases. However in 1987, the role of genetic factors was not known in humans. Although the
condition was usually dismissed as sporadic, numerous families with multiple affected
members have been observed.
The role of atrial natriuretic peptide levels in the pathogenesis or progression of
idiopathic dilated cardiomyopathy was just beginning to be explored in 1987. Although Syrian
hamster studies did not suggest a genetic deficiency as the primary cause of dilated
cardiomyopathy in that model, it was thought possible that ANP production or levels were
somehow involved in how the myocardium responds in idiopathic dilated cardiomyopathy
patients.
DESIGN NARRATIVE:
Eligible patients were interviewed and asked whether any first-degree relatives were willing
to participate. If family members participated, a three generation pedigree was constructed
and first-degree relatives contacted. The relatives were interviewed for a medical history
and medical records from other institutions were reviewed. Each family member had a brief
cardiovascular examination, a 12-lead electrocardiogram and 2-dimensional, M-mode, and
Doppler echocardiogram. Blood was drawn for determination of atrial natriuretic peptide
(ANP) levels. The contributions of variability in age, sex, drug use, smoking, and other
concomitants to variability in ANP and echocardiographic data were estimated. After removing
these sources of variability, the strength of similarity among family members was assessed.
The relative contributions of genes and shared environments to the similarity among family
members were estimated.
Heterogeneity in the mean levels of echocardiographic indices and ANP levels, familial
aggregation and etiology of aggregation were assessed between families with familial
idiopathic dilated cardiomyopathy and families with non-familial idiopathic dilated
cardiomyopathy.
The study completion date listed in this record was obtained from the "End Date" entered in
the Protocol Registration and Results System (PRS) record.
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