Document Detail


The changing spectrum of arrhythmogenic (right ventricular) cardiomyopathy.
MedLine Citation:
PMID:  22476658     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a clinically and genetically heterogeneous heart muscle disorder associated with ventricular arrhythmias and risk of sudden death. The disease is heredo-familial, and mutations in desmosomal genes have been identified in about half of patients. Recent experimental models confirm this disease develops after birth due to progressive myocardial dystrophy. Genotype-phenotype correlations, including magnetic resonance and pathology studies on heart specimens, are currently demonstrating that the spectrum of the disease is wider than initially thought and usually referred to with the adjective "right ventricular", with the evidence of biventricular or even isolated left ventricular forms, so that it is increasingly identified simply as "arrhythmogenic cardiomyopathy". A revision of the diagnostic criteria encompassing familial, electrocardiographic, arrhythmic, morpho-functional and histopathologic findings, has been made to improve diagnostic sensitivity and specificity, in particular of the concealed forms and left-dominant subtypes of the disease. Experimental models are mandatory to gain an insight into the cascade of cellular and molecular events leading from gene defect to myocardial dystrophy in ARVC.
Authors:
Stefania Rizzo; Kalliopi Pilichou; Gaetano Thiene; Cristina Basso
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-4-4
Journal Detail:
Title:  Cell and tissue research     Volume:  -     ISSN:  1432-0878     ISO Abbreviation:  -     Publication Date:  2012 Apr 
Date Detail:
Created Date:  2012-4-5     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0417625     Medline TA:  Cell Tissue Res     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Department of Cardiac, Thoracic and Vascular Sciences, University of Padua Medical School, Via Gabelli 61, 35121, Padua, Italy.
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