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European Journal of Pediatrics

, Volume 168, Issue 9, pp 1107–1115 | Cite as

QT interval prolongation and risk for cardiac events in genotyped LQTS-index children

  • H. Wedekind
  • D. Burde
  • S. Zumhagen
  • V. Debus
  • G. Burkhardtsmaier
  • G. Mönnig
  • G. Breithardt
  • E. Schulze-Bahr
Original Paper

Abstract

Congenital long-QT syndrome (LQTS) is an inherited cardiac disorder with a disturbance in repolarization characterized by a prolonged QT interval on the surface electrocardiogram and life-threatening ventricular tachycardia. Publications from the International LQTS Registry have provided information that the cardiac risk may be influenced by gender, genotype, exposure to arrhythmia triggers, and previous cardiac events. In children, early-onset of disease, changes in life style, and medical treatment is a sensitive issue and significant, gender-related differences of a first life-threatening event were reported. Thus, we investigated the clinical features of a large genotyped population of LQTS-index children (age ≤16 years) upon a single-center experience and determined risk factors for symptoms. Of 83 children [mean corrected QT interval (QTc) 510 ± 74 ms], 89% had LQT1, -2, or -3. Nine patients (11%) were identified as having Jervell and Lange-Nielsen syndrome. Among symptomatic children (n = 51, 61%), syncope was the most prevalent symptom at initial presentation (49%); however, aborted cardiac arrest (ACA) occurred in 33% and sudden cardiac death (SCD) in 18%, respectively, as the initial manifestation. During a mean follow-up period of 5.9 ± 4.7 years, 31% of the children developed symptoms while on therapy (86% syncope, 9% ACA, 5% SCD). Statistical analyses of risk factors for cardiac events showed that the QTc >500 ms was a strong and significant predictor for cardiac events during follow-up (p = 0.02). Furthermore, a prior syncope [hazard ratio (HR), 4.05; 95% confidence interval (CI), 1.1 to 15.0; p = 0.03] or an ACA (HR, 11.7; 95% CI, 3.1 to 43.4; p = <0.001) identified children with an increased risk for recurrent cardiac events compared to asymptomatic LQT children. LQTS-index children manifest with a high percentage of severe symptoms. Among presently validated risk factors for LQTS, a QTc interval >500 ms and a history of prior syncope or ACA were strong predictors for recurrent cardiac events.

Keywords

Children Arrhythmias Genetics Long QT syndrome Treatment 

Notes

Acknowledgments

This work was supported in part by grants from the Dr. Adolf Schilling Foundation, Münster, Germany; the Deutsche Forschungsgemeinschaft, Bonn, Germany (SFB 656-C1, Schu1082/3-1, and 3-2); and the Fondation Leducq, Paris, France.

Conflict of interest

The authors declare that they have no conflicts of interest.

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Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • H. Wedekind
    • 1
    • 2
    • 5
  • D. Burde
    • 1
  • S. Zumhagen
    • 1
  • V. Debus
    • 3
  • G. Burkhardtsmaier
    • 4
  • G. Mönnig
    • 1
  • G. Breithardt
    • 1
    • 2
  • E. Schulze-Bahr
    • 1
    • 2
  1. 1.Department of Cardiology and AngiologyUniversity Hospital of MünsterMünsterGermany
  2. 2.Leibniz Institute for Arteriosclerosis Research at the University of MünsterMünsterGermany
  3. 3.Department of Pediatric CardiologyUniversity Hospital of MünsterMünsterGermany
  4. 4.Department of Medical Informatics and BiomathematicsUniversity Hospital of MünsterMünsterGermany
  5. 5.St. Marien-Hospital HammKlinik für Kardiologie und IntensivmedizinHammGermany

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