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Utility of serial 12-lead electrocardiograms in children with Marfan syndrome

Alisa A Arunamata1, Charles T Nguyen1, Scott R Ceresnak1

  • 1Department of Pediatrics,Division of Pediatric Cardiology,Stanford University School of Medicine,Palo Alto,CA,USA.

Cardiology in the Young
|July 5, 2018
PubMed

Insights

Serial electrocardiograms may not be routinely useful for all pediatric Marfan syndrome patients. Only those with a prolonged QTc interval at baseline may benefit from this cardiac screening.

Area of Science:

  • Cardiology
  • Genetics
  • Pediatrics

Background:

  • Marfan syndrome is a genetic disorder affecting connective tissue.
  • Cardiac complications are a major concern in Marfan syndrome.
  • Routine follow-up is essential for managing pediatric Marfan patients.

Purpose of the Study:

  • To evaluate the effectiveness of serial electrocardiograms (ECGs) in the routine follow-up of pediatric patients with Marfan syndrome.
  • To determine if ECG findings correlate with echocardiographic results in this population.

Main Methods:

  • Included were pediatric patients (<=18 years) meeting Ghent criteria for Marfan syndrome with concurrent ECG and echocardiogram.
  • Matched controls with normal cardiac evaluations were used for comparison.
  • Demographic, clinical, echocardiographic, and ECG data were collected and analyzed.

Main Results:

  • Marfan patients showed higher frequencies of left atrial enlargement and left ventricular hypertrophy on ECGs compared to controls.
  • However, these ECG findings were largely unconfirmed by echocardiography.
  • Marfan patients had a longer QTc interval, with some exhibiting borderline prolonged QTc.

Conclusions:

  • Routine serial ECGs may not be beneficial for all asymptomatic pediatric Marfan patients.
  • ECG findings of left atrial enlargement and left ventricular hypertrophy lack echocardiographic support in this cohort.
  • Serial ECGs might be more appropriate for a specific subgroup of Marfan patients with a baseline prolonged QTc interval.
Abstract

Keywords:
Marfan syndromeelectrocardiogramoutpatient paediatric cardiologypaediatric echocardiogram

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