Risk stratification in hypertrophic cardiomyopathy

Détails

ID Serval
serval:BIB_FF8A24C9EE93
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Institution
Titre
Risk stratification in hypertrophic cardiomyopathy
Périodique
Current Opinion in Cardiology
Auteur(s)
Vassalli  G., Seiler  C., Hess  O. M.
ISSN
0268-4705 (Print)
Statut éditorial
Publié
Date de publication
05/1994
Volume
9
Numéro
3
Pages
330-6
Notes
Journal Article
Review --- Old month value: May
Résumé
Hypertrophic cardiomyopathy is a primary myocardial disorder with an autosomal pattern of inheritance, characterized by asymmetric left ventricular hypertrophy with myocyte and myofibrillar disarray. Approximately 30% to 50% of all cases are accounted for by mutations in the beta-cardiac myosin heavy chain gene on chromosome 14q1. Recent linkage analysis led to the association of the disease with additional loci on chromosomes 1q3, 11p13-q13, and 15q2, but the underlying gene defects are as yet unidentified. To date, about 34 mutations of the beta-cardiac myosin heavy chain gene have been described and shown to have important prognostic implications. Definite genotype-phenotype correlations have been described; however, wide diversity in cardiac morphology, pathophysiologic features, and clinical manifestations is still evident, even within the same family. The disease has an annual mortality of approximately 3%, related to both progressive heart failure and sudden cardiac death. Not only diastolic but also progressive systolic dysfunction with cavity dilatation occurs in a minority of patients with severe left ventricular hypertrophy. These patients usually have a poor prognosis, especially when atrial fibrillation ensues. Sudden death often occurs in young, asymptomatic or mildly symptomatic patients. The degree of hypertrophy and the presence of a pressure gradient are of little prognostic significance. Nonsustained ventricular tachycardia is associated with a poor prognosis in the presence of a history of syncope.
Mots-clé
Arrhythmia/genetics/mortality/pathology/physiopathology Cardiomyopathy, Hypertrophic/*genetics/mortality/pathology/physiopathology Chromosome Aberrations/genetics Chromosome Disorders Hemodynamic Processes/physiology Humans Myocardium/pathology Risk Factors Survival Rate
Pubmed
Web of science
Création de la notice
28/01/2008 10:32
Dernière modification de la notice
20/08/2019 16:29
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