Mitral valve surgery in children with the Marfan syndrome

Mitral valve disease is a common feature of the Marfan syndrome, seen in approximately 80% and 60% of adult and pediatric cases, respectively. Progressive mitral valve dysfunction with secondary left ventricular failure is the most common cause of cardiovascular morbidity and mortality in affected infants. The characteristic pathologic changes include annular dilatation, fibromyxomatous and nodular thickening of the leaflets, leaflet fenestration, and elongation and rupture of the chordae tendinae. Indications for surgical intervention include congestive heart failure, progressive chamber dilatation, atrial fibrillation, pulmonary hypertension, moderate mitral regurgitation, or, rarely, endocarditis. In addition to valve replacement, standard surgical repair techniques are effective in the management of the Marfan mitral valve. Mitral valve repair can often be performed coincident with aortic root replacement. Operative and mid-term follow-up results strongly suggest that current surgical techniques provide a safe and effective means to manage mitral valve dysfunction in pediatric patients with the Marfan syndrome.