The problem, challenge and opportunity of genetic heterogeneity in monogenic diseases predisposing to sudden death

Journal of the American College of Cardiology

Volumn 40, Issue 2, 2002, Pages 357-359

  Roden, Dan M ^a  

^a VANDERBILT UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

SODIUM CHANNEL;

BRUGADA SYNDROME; GENE MUTATION; GENETIC HETEROGENEITY; HEART ARRHYTHMIA; HUMAN; LONG QT SYNDROME; MONOGENIC DISORDER; PRIORITY JOURNAL; SHORT SURVEY; SUDDEN DEATH;

EID: 0037125409     PISSN: 07351097     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0735-1097(02)01963-0     Document Type: Editorial

References (35)

1. Genotype-phenotype relationship in Brugada syndrome: Electrocardiographic features differentiate SCN5A-related patients from non-SCN5A-related patients
2. Molecular and cellular mechanisms of cardiac arrhythmias
3. Low penetrance in the long-QT syndrome: Clinical impact
4. KVLQT1 C-terminal missense mutation causes a forme fruste long-QT syndrome
5. Evidence for a cardiac ion channel mutation underlying drug-induced QT prolongation and life-threatening arrhythmias
6. A common polymorphism associated with antibiotic-induced cardiac arrhythmia
7. Allelic variants in long QT disease genes in patients with drug-associated torsades de pointes
8. Kr potassium channel
9. The potential for prolongation and pro-arrhythmia by non-anti-arrhythmic drugs: Clinical and regulatory implications: Report on a policy conference of the European Society of Cardiology
10. Increased risk of arrhythmic events in long-QT syndrome with mutations in the pore region of the human ether-a-go-go-related gene potassium channel
11. Genotype-phenotype correlation in the long-QT syndrome: Gene-specific triggers for life-threatening arrhythmias
12. Influence of the genotype on the clinical course of the long-QT syndrome
13. + channel blockade and to increases in heart rate: Implications for gene-specific therapy
14. Sodium channel block with mexiletine is effective in reducing dispersion of repolarization and preventing torsade des pointes in LQT2 and LQT3 models of the long-QT syndrome
15. Ventricular fibrillation without apparent heart disease: Description of six cases
16. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: A distinct clinical and electrocardiographic syndrome: A multicenter report
17. "Brugada" syndrome: Clinical data and suggested pathophysiological mechanism
18. Genetic basis and molecular mechanism for idiopathic-ventricular fibrillation
19. A single Na(+) channel mutation causing both long-QT and Brugada syndromes
20. Clinical and molecular heterogeneity in the Brugada syndrome: A novel gene locus on chromosome 3
21. Cardiac conduction defects associate with mutations in SCN5A
22. Novel arrhythmogenic mechanism revealed by a long-QT syndrome mutation in the cardiac Na(+) channel
23. Novel mechanism for Brugada syndrome: Defective surface localization of an SCN5A mutant (R1432G)
24. Enhanced Na(+) channel intermediate inactivation in Brugada syndrome
25. Inherited Brugada and LQT-3 syndrome mutations of a single residue of the cardiac sodium channel confer distinct channel and clinical phenotypes
26. Two distinct congenital arrhythmias evoked by a multidysfunctional Na(+) channel
27. An SCN5A mutation causing isolated conduction disease is modulated by a common polymorphism
28. Flecainide-induced arrhythmia in canine ventricular epicardium. Phase 2 re-entry?
29. Taking the idio out of idiosyncratic-predicting torsades de pointes
30. Family history as a risk factor for primary cardiac arrest
31. Predicting sudden death in the population: The Paris prospective study I
32. Preliminary report: Effect of encainide and flecainide on mortality in a randomized trial of arrhythmia suppression after myocardial infarction
33. Increased risk of death and cardiac artest from encainide and flecainide in patients after non-Q-wave acute myocardial infarction in the Cardiac Arrhythmia Suppression trial
34. Differences in the electrophysiological response of canine ventricular epicardium and endocardium to ischemia: Role of the transient outward current
35. Sudden cardiac death, genes, and arrhythmogenesis: Consideration of new population and mechanistic approaches from a national heart, lung, and blood institute workshop, part i