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

BACKGROUND-: Mitral valve prolapse (MVP) is common in children with Marfan syndrome (MFS) and is associated with varying degrees of mitral regurgitation. However, the 3-dimensional (3D) morphology of the mitral valve (MV) in children with MFS and its relation to degree of mitral regurgitation (MR) are not known. The goals of this study were to describe the 3D morphology of the MV in children with MFS and to compare it to that of normal children. METHODS-: 3D transthoracic echocardiography was performed in 27 patients (age 3–21 years) meeting revised Ghent criteria for MFS as well as 27 normal children matched by age (±1 year). The 3D geometry of the MV apparatus in mid-systole was measured, and its association with clinical and 2D echocardiographic parameters was examined. RESULTS-: Compared to age-matched controls, children with MFS had larger 3D annular area (p