Risk stratification in the long-QT syndrome

New England Journal of Medicine

Volumn 348, Issue 19, 2003, Pages 1866-1874

  Priori, Silvia G ^a,c   Schwartz, Peter J ^b,c   Napolitano, Carlo ^a   Bloise, Raffaella ^a   Ronchetti, Elena ^a   Grillo, Massimiliano ^a   Vicentini, Alessandro ^a   Spazzolini, Carla ^b   Nastoli, Janni ^a   Bottelli, Georgia ^a   Folli, Roberta ^a   Cappelletti, Donata ^a  

^a IRCCS   (Italy)

^b FONDAZIONE IRCCS POLICLINICO SAN MATTEO   (Italy)

^c UNIVERSITY OF PAVIA   (Italy)

Author keywords

[No Author keywords available]

Indexed keywords

POTASSIUM CHANNEL; SODIUM CHANNEL;

ADULT; ARTICLE; CONTROLLED STUDY; DISEASE ACTIVITY; DISEASE COURSE; FEMALE; GENE LOCUS; GENE MUTATION; GENETIC ANALYSIS; GENOTYPE; HEART ARREST; HEART RATE; HIGH RISK POPULATION; HUMAN; INCIDENCE; LONG QT SYNDROME; MAJOR CLINICAL STUDY; MALE; MULTIVARIATE ANALYSIS; PHENOTYPE; PRIORITY JOURNAL; RISK ASSESSMENT; SEX DIFFERENCE; SUDDEN DEATH; SURVIVAL RATE; SYNCOPE;

ADULT; AGE OF ONSET; DISEASE-FREE SURVIVAL; ELECTROCARDIOGRAPHY; FEMALE; GENOTYPE; HEART ARREST; HUMANS; LONG QT SYNDROME; MALE; MULTIVARIATE ANALYSIS; MUTATION; PHENOTYPE; POTASSIUM CHANNELS; RISK ASSESSMENT; SODIUM CHANNELS;

EID: 0038415858     PISSN: 00284793     EISSN: None     Source Type: Journal    
DOI: 10.1056/NEJMoa022147     Document Type: Article

References (19)

1. Schwartz PJ, Priori SG, Napolitano C. The long QT syndrome. In: Zipes DP, Jalife J, eds. Cardiac electrophysiology: from cell to bedside. 3rd ed. Philadelphia: W.B. Saunders, 2000:597-615.
2. Romano C, Gemme G, Pongiglione R. Aritmie cardiache rare dell'età pediatrica. Clin Pediat (Bologna) 1963;45:656-83.
3. Ward OC. A new familial cardiac syndrome in children. J Irish Med Assoc 1964;54:103-6.
4. Keating MT, Sanguinetti MC. Molecular and cellular mechanisms of cardiac arrhythmias. Cell 2001;104:569-80.
5. Priori SG, Barhanin J, Hauer RNW, et al. Genetic and molecular basis of cardiac arrhythmias: impact on clinical management. Circulation 1999;99:518-28.
6. + channel blockade and to increases in heart rate: implications for gene-specific therapy. Circulation 1995;92:3381-6.
7. Moss AJ, Zareba W, Benhorin J, et al. ECG T-wave patterns in genetically distinct forms of the hereditary long QT syndrome. Circulation 1995;92:2929-34.
8. Zareba W, Moss AJ, Schwartz PJ, et al. Influence of the genotype on the clinical course of the long-QT syndrome. N Engl J Med 1998;339:960-5.
9. Locati EH, Zareba W, Moss AJ, et al. Age and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome: findings from the International LQTS Registry. Circulation 1998;97:2237-44.
10. Schwartz PJ, Priori SG, Spazzolini C, et al. Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation 2001;103:89-95.
11. Splawski I, Shen J, Timothy KW, et al. Spectrum of mutations in long-QT syndrome genes: KVLQT1, HERG, SCN5A, KCNE1, and KCNE2. Circulation 2000;102:1178-85.
12. Priori SG, Napolitano C, Schwartz PJ. Low penetrance in the long-QT syndrome: clinical impact. Circulation 1999;99:529-33.
13. Schwartz PJ. The idiopathic long QT syndrome: the need for a prospective registry. Eur Heart J 1983;4:529-31.
14. Moss AJ, Schwartz PJ, Crampton RS, Locati E, Carleen E. The long QT syndrome: a prospective international study. Circulation 1985;71:17-21.
15. Moss AJ, Schwartz PJ, Crampton RS, et al. The long QT syndrome: prospective longitudinal study of 328 families. Circulation 1991;84:1136-44.
16. Priori SG, Aliot E, Blomstrom-Lundqvist C, et al. Task Force on Sudden Cardiac Death of the European Society of Cardiology. Eur Heart J 2001;22:1374-450. [Erratum, Eur Heart J 2002;23:257.]
17. Moss AJ, Zareba W, Hall WJ, et al. Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation 2000;101:616-23.
18. Donger C, Denjoy I, Berthet M, et al. KVLQT1 C-terminal missense mutation causes a forme fruste long-QT syndrome. Circulation 1997;96:2778-81.
19. Moss AJ, Zareba W, Kaufman ES, et al. 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. Circulation 2002;105:794-9.