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Ventricular preexcitation in hypertrophic cardiomyopathy: Coincidence, clue, or concern?

Ahmet Korkmaz1, Ozcan Ozeke1, Serkan Topaloglu1

  • 1University of Health Sciences, Ankara Bilkent City Hospital, Department of Cardiology, Ankara, Turkey.

Journal of Electrocardiology
|September 18, 2025
PubMed

Insights

Unexplained left ventricular hypertrophy (LVH) may suggest hypertrophic cardiomyopathy (HCM). However, ventricular preexcitation in an LVH phenocopy warrants investigation for storage, metabolic, or syndromic disorders.

Area of Science:

  • Cardiology
  • Genetics
  • Pediatric Medicine

Background:

  • Unexplained left ventricular hypertrophy (LVH) is often initially diagnosed as hypertrophic cardiomyopathy (HCM), a genetic disorder affecting sarcomeric proteins.
  • Ventricular preexcitation is a known association with HCM.

Purpose of the Study:

  • To highlight the importance of considering non-HCM etiologies in young patients presenting with LVH and ventricular preexcitation.

Main Methods:

  • Clinical case review and differential diagnosis exploration.
  • Analysis of patient presentation including LVH and ventricular preexcitation.

Main Results:

  • Ventricular preexcitation in a young patient with an LVH phenocopy (not confirmed HCM) should prompt further investigation.
  • This presentation may indicate an underlying storage, metabolic, or syndromic condition rather than primary HCM.

Conclusions:

  • An HCM phenocopy with ventricular preexcitation necessitates a broader differential diagnosis beyond genetic HCM.
  • Early identification of underlying storage, metabolic, or syndromic conditions is crucial for appropriate management in pediatric cardiology.

Keywords:
Hypertrophic cardiomyopathyLeft ventricular hypertrophyPRKGA2 diseaseVentricular preexcitation

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