Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome: Findings from the international LQTS registry

Circulation

Volumn 97, Issue 22, 1998, Pages 2237-2244

  Locati, Emanuela H ^a,c,i   Zareba, Wojciech ^a   Moss, Arthur J ^a   Schwartz, Peter J ^c,d,e   Michael Vincent, G ^f   Lehmann, Michael H ^g   Towbin, Jeffrey A ^h   Priori, Silvia G ^c,d,e   Napolitano, Carlo ^c,d,e   Robinson, Jennifer L ^a   Andrews, Mark ^a   Timothy, Katherine ^f   Hall, W Jackson ^b  

^a UNIVERSITY OF ROCHESTER MEDICAL CENTER   (United States)

^b UNIVERSITY OF ROCHESTER   (United States)

^c UNIVERSITY OF MILAN   (Italy)

^d UNIVERSITY OF PAVIA   (Italy)

^e FONDAZIONE IRCCS POLICLINICO SAN MATTEO   (Italy)

^f UNIVERSITY OF UTAH SCHOOL OF MEDICINE   (United States)

^g SINAI GRACE HOSPITAL   (United States)

^h TEXAS CHILDREN S HOSPITAL   (United States)

ⁱ UNIVERSITY OF PERUGIA   (Italy)

Author keywords

Death, sudden; Genes; Long QT syndrome; Sex; Syncope

Indexed keywords

ADOLESCENT; ADULT; AGE; ARTICLE; CHILD; CLINICAL FEATURE; CONTROLLED STUDY; FAMILIAL DISEASE; FEMALE; GENE MUTATION; HEART VENTRICLE TACHYCARDIA; HETEROZYGOTE; HUMAN; LONG QT SYNDROME; MAJOR CLINICAL STUDY; MALE; PRIORITY JOURNAL; REGISTER; SEX DIFFERENCE; SUDDEN DEATH; SYNCOPE;

EID: 0032499656     PISSN: 00097322     EISSN: None     Source Type: Journal    
DOI: 10.1161/01.CIR.97.22.2237     Document Type: Article

References (43)

1. Romano C, Gemme G, Pongiglione R, Aritmie rare in età pediatrica. Clin Pediatr. 1963;45:656-683.
2. Ward OC. New familial cardiac syndrome in children. J Irish Med Assoc. 1964;54:103-106.
3. Jervell A, Lange-Nielsen F. Congenital sordo-mutism. functional heart disease with prolongation of the Q-T interval and sudden death. Am Heart J. 1957;54:59-68.
4. Schwanz PJ, Locati EH, Napoliuuio C, Priori SG, The idiopathic long QT syndrome. In: Zipes DP. Jalife J. eds. Cardiac Electrophysiology: From Cell to Bedside., 2nd ed. Philadelphia. Pa: WB Saunders Co: 1995;788-811.
5. Roden DM, Lazzara R, Rosen M, Schwanz PJ, Towbin J, Vincent M. Multiple mechanism in the long QT syndrome: current knowledge, gaps and future directions. Circulation, 1996;94:1996-2012.
6. Hashiba K. Hereditary QT prolongation syndrome in Japan: genetic analysis and pathological findings of the conduction system. Jpn Circ J. 1978;42:1133-1150.
7. Schwartz PJ, Periti M, Malliani A. The long QT syndrome. Am Heart J. 1975;89:378-390.
8. Moss AJ, Schwanz PJ, Crampton RS, Locati E, Carleen E. The long QT syndrome: a prospective international study. Circulation. 1985;71:17-21.
9. Moss AJ, Schwanz PJ, Crampton RS, Tzivoni D, Locati E, McCluer J, Hall WJ, Weitkemp L, Vincent GM, Carson A, Robinson J, Benhorin J. The long QT syndrome: prospective longitudinal study of 328 families. Circulation. 1991;84:1136-1144.
10. Zareba W, Moss AJ, LeCessie S, Locati EH, Robinson JL, Hall WJ, Andrews ML. Risk of cardiac events in family members of patients with long QT syndrome. J Am Coll Cardiol. 1995;26:1685-1691.
11. Schwanz PJ, Moss AJ, Vincent GM, Crampton RS. Diagnostic criteria for the long QT syndrome: an update. Circulation. 1993;88:782-784.
12. Merri M, Benhorin J, Alberti M, Locati EH, Moss AJ, Electrocardiographic quantitation of ventricular repolarization. Circulation. 1989;80:1301-1308.
13. Rautaharju PM, Zhou SH, Wong S, Calhoun HP, Berenson G Prineas R, Davignon A. Sex differences in the evolution of electrocardiographic QT interval with age. Can J Cardiol. 1992;8:690-695.
14. Stramba-Badiale M, Spagnolo D, Bosi G, Schwanz PJ. Are gender differences in QTc present at birth? Am J Cardiol. 1995;75:1277-1278.
15. Vincent GM, Timothy KW, Leppert M, Keating MT. The spectrum of symptoms and QT intervals in carriers of the gene for the long QT syndrome. N Engl J Med. 1992;327:846-852.
16. Towbin JA, Li H, Taggart RT, Lehmann MH, Schwanz PJ, Satler CA, Ayyagari R, Robinson JL, Moss AJ, Hejtmancik JF. Evidence of genetic heterogeneity in Romano-Ward long QT syndrome: analysis of 23 families. Circulation. 1994;90:2635-2644.
17. Keating MT, Atkinson D, Dunn C, Timothy K, Vincent GM, Leppert M. Linkage of a cardiac arrhythmia, the long QT syndrome, and Harvey ras-1 gene. Science. 1991;252:704-706.
18. Wang Q, Curran ME, Splawski I, Bum TC, Millholland JM, VanRaay TJ, Shen J, Timothy KV, Vincent GM, de Jager T, Schwanz PJ, Towbin JA, Moss AJ, Atkinson DL, Landes GM, Connors TD, Keating MT. Positional cloning of a novel potassium channel gene: KVLQT1 mutations cause cardiac arrhythmias. Nat Genet. 1996;12:17-23.
19. Curran ME, Splawski I, Timothy K, Vincent GM, Green ED, Keating MT. A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome. Cell. 1995;80:795-803.
20. Jiang C, Atkinson D, Towbin JA, Splawski I, Lehmann MH, Li H, Timothy K, Taggart RT, Schwartz PJ, Vincent GM, Moss AJ, Keating MT. Two long QT syndrome loci map to chromosome 3 and 7 with evidence for further heterogeneity. Nat Genet. 1994;8:141-147.
21. Wang Q, Shen J, Atkinson D, Li Z, Robinson JL, Moss A, Towbin JA, Keating MT. SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome. Cell. 1995;80:805-811.
22. Wang Q, Shen J, Li Z, Timothy K, Vincent GM, Priori SG, Schwanz PJ. Cardiac sodium channel mutations in patients with long QT syndrome, an inherited cardiac arrhythmia. Hum Mol Gen. 1995;4:1603-1607.
23. Neyroud N, Tesson F, Denjoy I, Leibovici M, Donger C, Barhanin J, Fuare S, Gary F, Coumel P, Petit C, Schwanz K, Giucheney P. A novel mutation in the potassium channel gene KVLQT1 causes the Jervell and Lange-Nielsen cardioauditory syndrome. Nat Genet. 1997;15:186-189.
24. Schott JJ, Charpentier F, Peltier S, Foley P, Drouin E, Bonhaur JB, Donnely P, Verngaud G, Bachner L, Moisan JP, Le Marec H, Pascal O. Mapping of a new gene for LQTS syndrome. Am J Hum Genet. 1995;57:1114-1122.
25. Bazett HC. An analysis of time relations of electrocardiograms. Heart. 1920;7:353-367.
26. Kaplan EL, Meier P. Non-parametric estimation from incomplete observations. J Am Stat Assoc: 1958;53:457-481.
27. Cox DR. Regression models and life-tables. J Stat Soc. 1972;34:187-220.
28. Hashiba K. Sex differences in phenotypic manifestation and gene transmission in the Romano-Ward syndrome. Ann N Y Acad Sci. 1992;644:142-156.
29. Garson A, Dick MD, Fournier A, Gillette PC, Hamilton R, Kugler JD, Van Hare GF, Vetter V, Vick GW. The long QT syndrome in children: an international study of 287 patients. Circulation. 1993;87:1866-1872.
30. Lehmann MH, Timothy K, Frankovich U, Fromm B, Keatmg M, Locati E, Schwanz PJ, Moss AJ, Taggart RT, Towbin JA, Vincent GM. Age-sex influence on rate corrected QT interval and QT-heart rate relationship in families with genotypically characterized long QT syndrome. J Am Coll Cardiol. 1997;29:93-99.
31. Kadish AH. The effect of gender on cardiac electrophysiology and arrhythmias. In: Zipes DP, Jalife J, eds. Cardiac Electrophysiology: From Cell In Bedside. 2nd ed. Philadelphia, Pa: WB Saunders Co; 1995:1268-1275.
32. Burke JH, Golberger JJ, Ehlert FA, Knuse JT, Parker MA, Kadish AH. Gender differences in heart rate before and after autonomic blockade: evidence against an intrinsic gender effect. Am J Med. 1996;100:537-543.
33. Drici MD, Burklow TR, Vedanandam H, Glazer RI, Woosley RL. Sex hormones prolong the QT interval and downregulate potassium channel expression in the rabbit heart. Circulation. 1996;94:1471-1474.
34. Boyle M, MacLusky N, Naftolin F, Kaczmarek L. Hormonal regulation of K+ channel messenger RNA in rat myometrium during oestrus cycle and in pregnancy. Nature. 1987;330:373-375.
35. Tanno K, Benditt DG, Sakaguchi S. The effect of 17beta-estradiol on delayed outward potassium current. Circulation. 1996;94(suppl 1):I-529. Abstract.
36. Rashba E, Zareba W, Moss AJ, Hall WJ, Robinson J, Locati EH, Schwartz PJ, Vincent GM, Timothy K, Andrews M. Does pregnancy cause an increased risk of cardiac events in long QT syndrome patients? Circulation. 1996;94(suppl 1):I-203. Abstract.
37. Moss AJ, Zareba W, Benhorin J, Locati EH, Hall WJ, Robinson JL, Schwanz PJ, Towbin JA, Vincent GM, Lehmann M, Keating MT, MacCluer JW, Timothy KW. ECG T wave patterns in genetically distinct forms of the hereditary long QT syndrome. Circulation. 1995;92:2929-2934.
38. Schwanz PJ. The idiopathic long QT syndrome: progress and questions. Am Heart J. 1985;109:399-411.
39. Schwanz PJ, Priori SG, Locati EH, Napolitano C, Cantú F, Towbin AJ, Keating MT, Hammoude H, Brown AM, Chen LK, Cotasky TJ, Long QT syndrome patients with mutations on the SCN5A and HERO genes have differential responses to Na+ channel blockade and to increase in heart rate: implications for gene-specific therapy. Circulation. 1995;92:3381-3386.
40. Makkar RR, Fromm BS, Steinman RT, Meissner MD, Lehmann MH. Female gender is a risk factor for torsades de pointes associated with cardiovascular drugs. JAMA. 1993;270:2590-2597.
41. Locati EH, Maisonblanche P, Dejode P, Cauchemez B, Coumel P. Spontaneous sequences of onset of torsade de pointes in patients with prolonged repolarization; quantitative analysis of Holter recordings. J Am Coll Cardiol. 1995;25:1564-1575.
42. Jackman WM, Friday KJ, Anderson JL, Aliot EM, Clark M, Lazzara R. The long QT syndromes: a critical review, new clinical observations and a unifying hypothesis. Prog Cardiovasc Dis. 1988;31:115-172.
43. Consensus Statement of the Joint Steering Committees of UCARE and of IVF-US (Bardy GH, Bigger JT Jr, Borggrefe M, Camm AJ, Cobb LA, Ewy GA, Hauer RNW Cook KH, Lane RD, Lazzara R, Marcus FI, Muller JE, Myerburg RJ, Priori SG, Schwanz PJ, Touboul P, Verrier RL, Wellens HJJ, Zipes DP). Survival of out-of-hospital cardiac arrest with apparently normal heart: need for definition and standardized clinical evaluation. Circulation. 1997;95:265-272.