Document Detail

Frequency and phenotypes of familial dilated cardiomyopathy.
MedLine Citation:
PMID:  9426039     Owner:  NLM     Status:  MEDLINE    
OBJECTIVES: This prospective study was performed to analyze the frequency and clinical characteristics of idiopathic dilated cardiomyopathy (DCM). BACKGROUND: Despite several previous reports on families with DCM, most cases are still believed to be sporadic, and specific clinical findings of the familial form are not well defined. METHODS: In 445 consecutive patients with angiographically proven DCM, we obtained detailed family histories to construct pedigrees and examined 970 first- and second-degree family members. RESULTS: Familial DCM was confirmed in 48 (10.8%) of the 445 index patients and was suspected in 108 (24.2%). The 156 patients with suspected or confirmed familial disease were younger at the time of diagnosis (p < 0.03) and more often revealed electrocardiographic changes (p = 0.0003) than patients with nonfamilial disease. Among the families of the 48 index patients with confirmed familial disease, five phenotypes of familial DCM could be identified: 1) DCM with muscular dystrophy; 2) juvenile DCM with a rapid progressive course in male relatives without muscular dystrophy; 3) DCM with segmental hypokinesia of the left ventricle; 4) DCM with conduction defects; and 5) DCM with sensorineural hearing loss. CONCLUSIONS: Up to 35% of patients with DCM may have an inherited disorder. Distinct clinical phenotypes can be observed in some families, suggesting a common molecular cause of the disease.
E Grünig; J A Tasman; H Kücherer; W Franz; W Kübler; H A Katus
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of the American College of Cardiology     Volume:  31     ISSN:  0735-1097     ISO Abbreviation:  J. Am. Coll. Cardiol.     Publication Date:  1998 Jan 
Date Detail:
Created Date:  1998-01-27     Completed Date:  1998-01-27     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8301365     Medline TA:  J Am Coll Cardiol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  186-94     Citation Subset:  AIM; IM    
University of Heidelberg, Medizinishce Klinik III, Germany.
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MeSH Terms
Cardiomyopathy, Dilated / complications,  genetics*,  ultrasonography
Disease Progression
Hearing Loss, Sensorineural / complications
Middle Aged
Muscular Dystrophies / complications
Prospective Studies
Risk Assessment
Comment In:
J Am Coll Cardiol. 1998 Oct;32(4):1135-6   [PMID:  9768747 ]

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