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[Clinical and genetic manifestations of left ventricular non-compaction in children]

Emanuele Monda1, Gaetano Diana2, Federica Verrillo2

  • 1Unità di Malattie Genetiche e Rare Cardiovascolari, Dipartimento di Scienze Mediche Traslazionali, Università della Campania "Luigi Vanvitelli, Ospedale Monaldi, Napoli - Institute of Cardiovascular Science, University College London, Londra, UK.

Giornale Italiano Di Cardiologia (2006)
|January 25, 2024
PubMed

Insights

Left ventricular non-compaction (LVNC) is a rare heart muscle disease increasingly diagnosed in children. This review details its clinical and genetic features and evaluates diagnostic criteria for pediatric cases.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Genetics

Background:

  • Left ventricular non-compaction (LVNC) is a myocardial disease with increasing prevalence in children.
  • Etiology is diverse, encompassing inherited and acquired conditions.
  • While often asymptomatic, LVNC can lead to heart failure, arrhythmias, and thromboembolic events.

Approach:

  • This review synthesizes current knowledge on pediatric LVNC.
  • It describes clinical and genetic characteristics.
  • It evaluates the utility of proposed diagnostic criteria in children.

Key Points:

  • LVNC presents a unique two-layered myocardial structure.
  • Diagnostic criteria for LVNC face limitations in pediatric application.
  • Understanding genetic factors is crucial for diagnosis and management.

Conclusions:

  • Further refinement of diagnostic criteria for pediatric LVNC is necessary.
  • Comprehensive assessment of clinical and genetic factors aids in managing LVNC in children.
  • Increased awareness and advanced imaging contribute to higher pediatric LVNC detection rates.

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