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Phenotype of Children with QT Prolongation Identified Using an Institution-Wide QT Alert System

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Abstract

QT prolongation is an independent risk factor for cardiovascular mortality in adults. However, there is little information available on pediatric patients with QT prolongation and their outcomes. Herein, we evaluated the prevalence of QT prolongation in pediatric patients identified by an institution-wide QT alert system, and the spectrum of their phenotype. Patients with documented QT prolongation on an ECG obtained between November 2010 and June 2011 were included. There were 1303 pediatric ECGs, and 68 children had electrographically isolated QT prolongation. Comprehensive review of medical records was performed with particular attention to QT-prolonging clinical, laboratory, and medication data, which were summarized into a pro-QTc score. Overall, 68 (5 %) pediatric patients had isolated QT prolongation. The mean age of this pediatric cohort was 9 ± 6 years, and the average QTc was 494 ± 42 ms. All children had 1 or more QT-prolonging risk factor(s), most commonly QT-prolonging medications. One patient was identified with congenital long QT syndrome (LQTS), which was not previously diagnosed. In one-year follow-up, only one pediatric death (non-cardiac) occurred (1.5 %). Potentially QT-offending/pro-arrhythmic medications were changed in 80 % of pediatric patients after the physician received the QT alert. Children with QT prolongation had very low mortality and minimal polypharmacy. Still, medications and other modifiable conditions were the most common causes of QT prolongation. Children with a prolonged QTc should be evaluated for modifiable QT-prolonging factors. However, if no risk factors are present or the QTc does not attenuate after risk factor modification/removal, the child should be evaluated for congenital LQTS.

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Abbreviations

ECG:

Electrocardiogram

LQTS:

Long QT syndrome

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Acknowledgments

This work was supported by the Mayo Clinic Windland Smith Rice Comprehensive Sudden Cardiac Death Program.

Ethical standard

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Conflict of interest

Dr. Ackerman is a consultant for Boston Scientific, Gilead Sciences, Medtronic, and St. Jude Medical. Dr. Ackerman and Mayo Clinic receive royalties from Transgenomic’s FAMILION-LQTS and FAMILION-CPVT genetic tests. The other authors have no conflicts of interest to disclose. None of the disclosures pertain to this study, and none of the companies provided financial support for this study.

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Authors and Affiliations

  1. Department of Pediatric and Adolescent Medicine/Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA

    Heather N. Anderson, J. Martijn Bos & Michael J. Ackerman

  2. Department of Molecular Pharmacology and Experimental Therapeutics/Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Guggenheim 501, 200 First Street SW, Rochester, MN, 55905, USA

    J. Martijn Bos, Kristina H. Haugaa & Michael J. Ackerman

  3. Department of Internal Medicine/Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA

    J. Martijn Bos & Michael J. Ackerman

  4. Department of Health Care Policy and Research, Mayo Clinic, Rochester, MN, USA

    Bruce W. Morlan

  5. Department of Statistics, Mayo Clinic, Rochester, MN, USA

    Robert F. Tarrell

  6. Department of Internal Medicine, Division of General Internal Medicine, Mayo Clinic, Rochester, MN, USA

    Pedro J. Caraballo

Authors
  1. Heather N. Anderson
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  2. J. Martijn Bos
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  3. Kristina H. Haugaa
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  4. Bruce W. Morlan
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  5. Robert F. Tarrell
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  6. Pedro J. Caraballo
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  7. Michael J. Ackerman
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Corresponding author

Correspondence to Michael J. Ackerman.

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Anderson, H.N., Bos, J.M., Haugaa, K.H. et al. Phenotype of Children with QT Prolongation Identified Using an Institution-Wide QT Alert System. Pediatr Cardiol 36, 1350–1356 (2015). https://doi.org/10.1007/s00246-015-1164-y

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  • DOI: https://doi.org/10.1007/s00246-015-1164-y

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