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Arrhythmias May Hide a Genetic Cardiomyopathy in Left Ventricular Hypertrabeculation in Children: A Single-Center

Irma Battipaglia1, Nicoletta Cantarutti1, Marianna Cicenia1

  • 1Pediatric Cardiology and Cardiac Arrhythmias Complex Unit, Bambino Gesù Children's Hospital IRCCS, 00050 Rome, Italy.

Children (Basel, Switzerland)
|October 26, 2024
PubMed

Insights

Left ventricular hypertrabeculation (LVHT) in children can cause arrhythmias in 23.6% of cases, though outcomes are generally good. Genetic variants are linked to these arrhythmic manifestations in pediatric LVHT patients.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Genetics

Background:

  • Left ventricular hypertrabeculation (LVHT) is a myocardial disorder with variable clinical presentations, ranging from asymptomatic to severe heart failure.
  • Distinguishing between benign and pathological forms of LVHT remains a clinical challenge.
  • Arrhythmias, thromboembolic events, and sudden cardiac death (SCD) are potential complications of LVHT.

Purpose of the Study:

  • To investigate the prevalence and types of arrhythmic manifestations in a large pediatric cohort with LVHT.
  • To correlate arrhythmic events with genetic findings and other clinical markers in pediatric patients with LVHT.
  • To assess the clinical outcomes in pediatric patients diagnosed with LVHT.

Main Methods:

  • Retrospective analysis of 140 pediatric patients diagnosed with LVHT between 2013 and 2023.
  • Collection of data on family history, instrumental examinations, cardiac magnetic resonance imaging, and genetic testing.
  • Evaluation of clinical outcomes, including the occurrence of arrhythmias and need for interventions like implantable cardioverter-defibrillators (ICDs).

Main Results:

  • Arrhythmias were identified in 33 (23.6%) pediatric patients, including supraventricular tachyarrhythmias, ventricular arrhythmias (VT, VF, PVCs), sinus node dysfunction, and atrioventricular blocks (AVB).
  • A significant association was found between the presence of arrhythmias and genetic variants of class 4 or 5 (p = 0.037).
  • Three patients required an implantable cardioverter-defibrillator (ICD); no cases of sudden cardiac death (SCD) were reported.

Conclusions:

  • Arrhythmias are a significant concern in pediatric patients with LVHT, occurring in nearly a quarter of the cohort.
  • Genetic variants may play a role in the development of arrhythmias in pediatric LVHT.
  • Despite the prevalence of arrhythmias, the overall outcomes in this pediatric cohort were favorable, with no reported SCD.
Abstract

Keywords:
arrhythmiascardiomyopathychildrenleft ventricular hypertrabeculationsudden cardiac death

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