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Three-Dimensional Mitral Valve Morphology in Children and Young Adults With Marfan Syndrome

Matthew A Jolley1, Peter E Hammer2, Sunil J Ghelani3

  • 1Department of Anesthesia and Critical Care Medicine and Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts.

Journal of the American Society of Echocardiography : Official Publication of the American Society of Echocardiography
|August 13, 2018
PubMed
Abstract

Insights

Children with Marfan syndrome (MFS) exhibit distinct three-dimensional mitral valve (MV) differences, including larger annular areas and greater billow volumes. These MV changes, particularly increased billow volume, are linked to mitral regurgitation (MR) severity in MFS patients.

Area of Science:

  • Cardiovascular Imaging
  • Pediatric Cardiology
  • Connective Tissue Disorders

Background:

  • Mitral valve (MV) prolapse and mitral regurgitation (MR) are common in children with Marfan syndrome (MFS).
  • The specific three-dimensional (3D) morphology of the MV in pediatric MFS and its correlation with MR severity remain largely uncharacterized.

Purpose of the Study:

  • To delineate the 3D morphology of the MV in children with MFS.
  • To compare the 3D MV geometry between children with MFS and healthy controls.
  • To investigate the relationship between 3D MV morphology and the degree of MR in MFS.

Main Methods:

  • Three-dimensional transthoracic echocardiography was utilized in 27 children with MFS and 27 age-matched healthy children.
  • Detailed 3D measurements of the MV apparatus in midsystole were performed.
  • Associations between 3D MV metrics and clinical/2D echocardiographic parameters were analyzed.

Main Results:

  • Children with MFS demonstrated significantly larger 3D annular areas, smaller annular height/commissural width ratios, greater leaflet billow volumes, and smaller tenting dimensions compared to controls.
  • Increased leaflet billow volume in MFS was strongly associated with moderate or severe MR.
  • The 3D metrics exhibited acceptable intra- and interuser variability.

Conclusions:

  • Pediatric MFS is characterized by flatter, more dilated MV annuli, increased billow volumes, and reduced tenting heights compared to normal.
  • Elevated billow volume is a key morphological feature associated with MR in MFS.
  • 3D MV quantification offers potential for identifying MFS and other connective tissue disorders, warranting further investigation into its clinical implications.
Keywords:
Echocardiography (three-dimensional)Marfan syndromeMitral regurgitationMitral valve

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