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Abnormal Mitral Valve Dimensions in Pediatric Patients with Hypertrophic Cardiomyopathy

Daryl Schantz1,2, Lee Benson1, Jonathan Windram3

  • 1Department of Paediatrics, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.

Pediatric Cardiology
|March 11, 2016
PubMed

Insights

Children and adolescents with hypertrophic cardiomyopathy (HCM) have significantly longer mitral valve leaflets compared to healthy children. This mitral valve elongation in HCM is not linked to outflow obstruction.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Genetics

Background:

  • Hypertrophic cardiomyopathy (HCM) involves cardiac structural abnormalities beyond myocardial thickening.
  • Adults with HCM exhibit longer mitral valve leaflets compared to controls.
  • Elongated mitral valve leaflets may be a characteristic feature of HCM.

Purpose of the Study:

  • To investigate if pediatric and adolescent patients with HCM also present with elongated mitral valve leaflets.
  • To compare mitral valve leaflet dimensions in pediatric HCM patients versus healthy controls.

Main Methods:

  • Retrospective review of clinical and echocardiographic data from 46 pediatric HCM patients.
  • Evaluation of cardiac magnetic resonance imaging studies.
  • Comparison of anterior and posterior mitral valve leaflet lengths between HCM patients and 20 healthy controls.

Main Results:

  • Patients with HCM showed significantly longer anterior mitral valve leaflets (28.4 ± 4.9 mm) than controls (25.2 ± 3.6 mm, p=0.013).
  • Posterior mitral valve leaflets were also longer in the HCM group (16.3 ± 3.0 mm) compared to controls (13.1 ± 2.3 mm, p<0.0001).
  • No correlation was found between leaflet length and left ventricular outflow tract gradient or systolic anterior motion.

Conclusions:

  • Pediatric and adolescent patients with HCM possess abnormally elongated mitral valve leaflets.
  • Mitral valve elongation in pediatric HCM does not appear to cause or result from left ventricular outflow obstruction.
  • The underlying mechanism for mitral valve elongation in HCM remains unclear and seems independent of hemodynamic factors.

Keywords:
Cardiac magnetic resonanceChildrenHypertrophic cardiomyopathyMitral valveMyocardial delayed enhancement

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