title: Molecular genetic basis of sudden cardiac death.
abstract: In this review, the up-to-date understanding of the molecular basis of disorders causing sudden death will be described. Two arrhythmic disorders causing sudden death have recently been well described at the molecular level, the long QT syndromes (LQTS) and Brugada syndrome, and in this article we will review the current scientific knowledge of each disease. A third disorder, hypertrophic cardiomyopathy (HCM), a myocardial disorder causing sudden death, has also been well studied. Finally, a disorder in which both myocardial abnormalities and rhythm abnormalities coexist, arrhythmogenic right ventricular dysplasia (ARVD) will also be described. The role of the pathologist in these studies will be highlighted.
authors.affiliation: Department of Pediatrics (Cardiology), Texas Children's Hospital and Baylor College of Medicine, One Baylor Plaza, Room 333E, Houston, TX 77030, USA. jtowbin@bcm.tmc.edu
authors.forename: J A
authors.initials: JA
authors.lastname: Towbin
mesh_terms: D000818:Animals; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D024741:Cardiomyopathy, Hypertrophic, Familial / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D016757:Death, Sudden, Cardiac / Q000209:etiology* / Q000473:pathology; D004285:Dogs; D020022:Genetic Predisposition to Disease; D006801:Humans; D007473:Ion Channels; D008133:Long QT Syndrome / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D008967:Molecular Biology; D014693:Ventricular Fibrillation / Q000235:genetics / Q000473:pathology / Q000503:physiopathology
chemical_list: D007473:Ion Channels