Clin Res Cardiol 101, Suppl 1, April 2012

P1023 - Functional remodeling of floppy mitral valve in children with Marfan Syndrome
E. M. Delmo Walter1, M. Hübler1, T. Komoda1, H. Siniawski1, R. Hetzer1
1Klinik für Herz-, Thorax- und Gefäßchirurgie, Deutsches Herzzentrum Berlin, Berlin;
Objective: Surgical treatment of severe mitral regurgitation caused by a floppy mitral valve in patients with Marfan syndrome is challenging because the underlying connective tissue disease might influence the durability of surgical repair. The aim of this study is to determine the mid- and long-term functional outcome of mitral valve repair for floppy mitral valves in children with Marfan syndrome.
Methods: Between 1997 and 2011, 6 Marfan children aged 5-15 years old, presented with severe mitral valve regurgitation with left ventricular dilatation. Intraoperative findings revealed enormously dilated annulus, enlarged anterior and posterior leaflets with prolapse of the posterior leaflets caused by chordal elongation in 3 patients and chordal rupture in another 3. Mitral repair using modified surgical repair techniques according to the presenting valve morphology was performed without using any prosthetic materials.
Results: There was neither morbidity nor mortality, nor any valve-related complications. The postoperative course was uneventful after each operation. On follow-up, there was either absent or minimal regurgitation except for one patient, who underwent a repeat mitral valve repair with concomitant ascending aortic root replacement 13 years after the initial repair.  At a mean follow up of 7.6 years, all patients were in NYHA class I or II, with improved functional capacity. 
Conclusion: Mid- and long-term outcome of functional remodeling of floppy mitral valve in Marfan children, accomplished by repair techniques without the use of any prosthetic materials, is durable and satisfactory. This suggests that the connective tissue defect did not compromise the results of mitral valve repair.
Clin Res Cardiol 101, Suppl 1, April 2012
Zitierung mit Vortrags- oder Posternummer s.o.
DOI 10.1007/s00392-012-1100-6