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Left Ventricular Noncompaction-A Systematic Review of Risk Factors in the Pediatric Population

Katarzyna Łuczak-Woźniak1, Bożena Werner2

  • 1Department of Pediatric Cardiology and General Pediatrics, Doctoral School, Medical University of Warsaw, 02-091 Warsaw, Poland.

Journal of Clinical Medicine
|April 3, 2021
PubMed

Insights

Left ventricular noncompaction (LVNC) in children involves various risk factors for poor outcomes. Echocardiography, ECG, cardiac MRI, and genetic testing aid in identifying high-risk pediatric patients needing closer monitoring.

Area of Science:

  • Cardiology
  • Genetics
  • Pediatric Medicine

Background:

  • Left ventricular noncompaction (LVNC) is a complex, often inherited cardiomyopathy with varied clinical presentations.
  • Understanding risk factors and diagnostic nuances in pediatric LVNC is critical for timely intervention.

Purpose of the Study:

  • To review risk factors for adverse outcomes in pediatric LVNC.
  • To discuss diagnostic modalities and differentiate pediatric from adult LVNC.
  • To identify children requiring heightened clinical attention.

Main Methods:

  • Systematic literature review of 1983 articles, with 23 meeting inclusion criteria.
  • Analysis of echocardiographic parameters (ejection fraction, dimensions, wall compaction, strains).
  • Evaluation of electrocardiogram (ECG) findings (T-wave, QRS-T angle, arrhythmia) and cardiac magnetic resonance (CMR) imaging (systolic dysfunction, late gadolinium enhancement).
  • Review of genetic testing associations with outcomes.

Main Results:

  • Echocardiographic findings like reduced ejection fraction and altered dimensions are linked to adverse outcomes in pediatric LVNC.
  • ECG abnormalities (T-wave changes, QRS-T angle) and arrhythmias indicate higher risk in children.
  • CMR effectively identifies systolic dysfunction and late gadolinium enhancement.
  • Specific gene mutations identified through genetic testing correlate with poorer prognoses.

Conclusions:

  • ECG and imaging (echocardiography, CMR) are vital for identifying risk factors and high-risk pediatric LVNC patients.
  • Genetic testing can aid in risk stratification for pediatric LVNC.
  • Refined diagnostic criteria are essential to prevent unnecessary activity restrictions in affected children.

Keywords:
adolescentschildrenhypertrabeculationleft ventricular noncompactionnoncompaction cardiomyopathy

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