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Breath Holding Spells in Children with Long QT Syndrome

Jeffrey A Robinson1, J Martijn Bos1,2, Susan P Etheridge3

  • 1Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minn, USA.

Congenital Heart Disease
|April 29, 2015
PubMed

Insights

Breath holding spells (BHS) in children with Long QT syndrome (LQTS) are rare, occurring in 4.3% of cases, primarily in LQT1. While not frequent, BHS can be the initial symptom of LQTS, necessitating careful evaluation.

Area of Science:

  • Pediatric Cardiology
  • Clinical Genetics
  • Cardiac Electrophysiology

Background:

  • Long QT syndrome (LQTS) is a genetic heart disorder.
  • Breath holding spells (BHS) are common in children.
  • Anecdotal evidence suggests a link between BHS and LQTS.

Purpose of the Study:

  • To determine the frequency of BHS in children diagnosed with LQTS.
  • To investigate BHS as a presenting symptom in pediatric LQTS.

Main Methods:

  • Retrospective review of 115 children diagnosed with LQTS at ≤5 years old.
  • Analysis of clinical presentation, LQTS symptoms, diagnostic tests, and treatments.
  • Follow-up duration averaged 6.4 years.

Main Results:

  • 4.3% of LQTS patients (5/115) experienced BHS.
  • All BHS cases occurred in patients with LQT1 (48% of cohort).
  • BHS presented as the initial symptom in 4.3% of symptomatic patients.

Conclusions:

  • BHS in children with LQTS are relatively rare.
  • BHS can be the presenting symptom of LQTS.
  • Distinguishing BHS from LQTS-triggered events is crucial for timely treatment.
Abstract

Keywords:
Breath Holding SpellsChildrenLong QT SyndromeSpellsTorsades de Pointes

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