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Mitral Annular Disjunction Associated with Ventricular Dilation in Pediatric Marfan Syndrome: A Cardiovascular

Ryan S Bishop1,2, Tam T Doan3,4, Natalie K Craik3,4

  • 1Division of Cardiology, Texas Children's Hospital, Baylor College of Medicine, 6651 Main Street MC-E1920, Houston, TX, 77030, USA. ryan.bishop@bcm.edu.

Pediatric Cardiology
|October 28, 2024
PubMed
Abstract

Insights

Mitral annular disjunction (MAD) is linked to left ventricular (LV) dilation in pediatric Marfan syndrome (MFS) patients, independent of mitral valve prolapse. This finding highlights MAD as a key indicator of cardiac changes in young MFS individuals.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Cardiovascular Imaging

Background:

  • Mitral annular disjunction (MAD) is an emerging marker for adverse cardiovascular events in Marfan syndrome (MFS).
  • Previous adult studies suggest a link between MFS, left ventricular (LV) dilation, and reduced ejection fraction (LVEF).

Purpose of the Study:

  • To investigate the association between MAD and LV dilation in pediatric MFS patients.
  • To determine if MAD is an independent predictor of LV dilation in this population.

Main Methods:

  • Retrospective analysis of pediatric MFS patients (<19 years) using cardiac MRI (CMR) or echocardiography.
  • Assessment of MAD and mitral valve prolapse (MVP).
  • Measurement of LV volumes (LVEDV, LVESV), indexed volumes, z-scores, and LVEF, excluding significant volume load from regurgitation.

Main Results:

  • MAD was present in 66.7% of the 42 analyzed pediatric MFS patients.
  • Significant LV dilation (LVESV z-score > 2) was observed in 42.9% of patients.
  • Multivariable analysis confirmed MAD as an independent predictor of elevated LVESV z-score (RR 3.88, p=0.046), even after accounting for MVP.

Conclusions:

  • Mitral annular disjunction is associated with significant left ventricular dilation in pediatric Marfan syndrome patients.
  • This association appears independent of volume overload from mitral or aortic regurgitation.
  • Further prospective research is warranted to understand the long-term implications of MAD on LV remodeling in pediatric MFS.
Keywords:
CardiomyopathyMarfan syndromeMitral annular disjunctionMitral valve prolapse

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