Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome

Findings from the international LQTS registry

Emanuela H. Locati, Wojciech Zareba, Arthur J. Moss, Peter J. Schwartz, G. Michael Vincent, Michael H. Lehmann, Jeffrey A. Towbin, Silvia G. Priori, Carlo Napolitano, Jennifer L. Robinson, Mark Andrews, Katherine Timothy, W. Jackson Hall

Research output: Contribution to journalArticle

364 Citations (Scopus)

Abstract

Background: Unexplained female predominance is observed in long-QT syndrome (LQTS), a congenital autosomal disorder with prolonged repolarization and syncope or sudden death due to ventricular tachyarrhythmias. Our objectives were to evaluate age- and sex-related differences in events among LQTS patients referred to the LQTS International Registry. Methods and Results: Age- and sex-related occurrence of events was analyzed in 479 probands (70% females) and 1041 affected family members (QT(c) >440 ms, 58% females). LQTS-gene mutations were identified in 162 patients: 69 LQT1 carriers (KVLQT1 on 11p15.5), 62 LQT2 carriers (HERG on 7q35-36), and 31 LQT3 carriers (SCN5A on 3p21-24). Females predominated among 366 probands (71% females) and 230 symptomatic family members (62% females). Male probands were younger than females at first event (8±7 versus 14±10 years, P

Original languageEnglish
Pages (from-to)2237-2244
Number of pages8
JournalCirculation
Volume97
Issue number22
Publication statusPublished - Jun 9 1998

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Long QT Syndrome
Sex Characteristics
Registries
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Syncope
Sudden Death
Tachycardia
Mutation
Genes

Keywords

  • Death, sudden
  • Genes
  • Long-QT syndrome
  • Sex
  • Syncope

ASJC Scopus subject areas

  • Physiology
  • Cardiology and Cardiovascular Medicine

Cite this

Locati, E. H., Zareba, W., Moss, A. J., Schwartz, P. J., Michael Vincent, G., Lehmann, M. H., ... Hall, W. J. (1998). Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome: Findings from the international LQTS registry. Circulation, 97(22), 2237-2244.

Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome : Findings from the international LQTS registry. / Locati, Emanuela H.; Zareba, Wojciech; Moss, Arthur J.; Schwartz, Peter J.; Michael Vincent, G.; Lehmann, Michael H.; Towbin, Jeffrey A.; Priori, Silvia G.; Napolitano, Carlo; Robinson, Jennifer L.; Andrews, Mark; Timothy, Katherine; Hall, W. Jackson.

In: Circulation, Vol. 97, No. 22, 09.06.1998, p. 2237-2244.

Research output: Contribution to journalArticle

Locati, EH, Zareba, W, Moss, AJ, Schwartz, PJ, Michael Vincent, G, Lehmann, MH, Towbin, JA, Priori, SG, Napolitano, C, Robinson, JL, Andrews, M, Timothy, K & Hall, WJ 1998, 'Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome: Findings from the international LQTS registry', Circulation, vol. 97, no. 22, pp. 2237-2244.
Locati, Emanuela H. ; Zareba, Wojciech ; Moss, Arthur J. ; Schwartz, Peter J. ; Michael Vincent, G. ; Lehmann, Michael H. ; Towbin, Jeffrey A. ; Priori, Silvia G. ; Napolitano, Carlo ; Robinson, Jennifer L. ; Andrews, Mark ; Timothy, Katherine ; Hall, W. Jackson. / Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome : Findings from the international LQTS registry. In: Circulation. 1998 ; Vol. 97, No. 22. pp. 2237-2244.
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