Assessment of mitral valve function in children and young adults with hypertrophic cardiomyopathy using

Navya Joseph1, Mary Craft2, LuAnn Mill1

  • 1University of Nebraska Medical Center, Omaha, NE, United States.

Insights

Real-time 3D echocardiography effectively assesses papillary muscle and mitral valve structure in pediatric hypertrophic cardiomyopathy (HCM). Left ventricular mass, not PM mass, correlated with adverse outcomes in young HCM patients.

Area of Science:

  • Cardiology
  • Medical Imaging
  • Pediatric Cardiology

Background:

  • Hypertrophic cardiomyopathy (HCM) is a genetic heart disease characterized by left ventricular hypertrophy.
  • Assessing structural and functional changes in papillary muscles (PM) and the mitral valve (MV) is crucial for understanding HCM progression and outcomes, especially in pediatric populations.

Purpose of the Study:

  • To evaluate the structure and function of papillary muscles (PM) and mitral valve (MV) in children and young adults with mild to moderate hypertrophic cardiomyopathy (HCM).
  • To correlate these cardiac parameters with adverse outcomes in HCM patients using real-time three-dimensional echocardiography (3DE).

Main Methods:

  • Transthoracic real-time 3DE was performed on 20 HCM patients and 20 age- and gender-matched controls.
  • Measurements included left ventricular (LV) mass, PM mass, and apical displacement of the anterolateral PM (APL index).
  • MV annulus and leaflet structure and function were analyzed; all 3DE measurements were correlated with adverse events.

Main Results:

  • HCM patients exhibited significantly increased LV mass and PM mass compared to controls (p < 0.001).
  • The APL index was significantly lower in HCM patients (0.44 ± 0.07) than in controls (0.55 ± 0.04, p < 0.001).
  • LV lateral wall length and LV mass correlated with adverse HCM outcomes, whereas the APL index and total PM mass did not.

Conclusions:

  • 3DE is a feasible tool for assessing PM and MV geometry in pediatric and young adult HCM patients.
  • Morphologic and functional changes in the anterolateral PM can occur independently of MV annulus changes.
  • Further prospective studies are needed to validate LV lateral wall length and LV mass as predictors of adverse events in HCM.
Abstract

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