Floppy Mitral Valve in Children with Marfan Syndrome

Purpose: Surgical treatment of floppy mitral valve in Marfan syndrome is challenging because the connective tissue disease might influence durability of repair. Enormous annular size and extensive pathological tissue involvement makes repair highly demanding. This study evaluates the long-term outcome of repair for floppy mitral valves in children with Marfan syndrome.

Methods: Between 1997 and 2014, eleven children with Marfan’s syndrome (mean age, 10.2, range: 0.58–16.8) years presented with severe mitral valve regurgitation with left ventricular dilatation. Four patients had associated ascending aortic aneurysm with annular dilatation and aortic valve regurgitation while another had ascending root aneurysm, severe tricuspid regurgitation and pectus excavatum. Simultaneous correction of all presenting pathologies was performed including correction of the chest wall deformity. Intraoperative mitral valve findings revealed enormously dilated annulus (n = 4), enlarged anterior and posterior leaflets (n = 3) with prolapse of the posterior leaflets caused by chordal elongation in three patients and chordal rupture in another three. Mitral valve repair using modified surgical repair techniques including partial obliteration of the mitral valve orifice, leaflet plication and annulus stabilization with autologous pericardium according to the presenting valve morphology was performed.

Results: There was neither morbidity nor mortality, nor any valve-related complications. The postoperative course was uneventful after each operation. During a median duration of follow-up of 9.9 (range: 4.3–23.2) years, there was either absent or minimal regurgitation except in one patient, who underwent a repeat mitral valve repair with concomitant ascending aortic root replacement 13 years after the initial repair. All patients were in NYHA class I or II, with improved functional capacity.

Conclusion: Midterm and long-term outcome of repair of floppy mitral valve in children with Marfan syndrome, accomplished by repair techniques without the use of any prosthetic materials, is durable and satisfactory. This suggests that the connective tissue disease at least during the period of follow-up does not compromise the results of mitral valve repair.