Ann Thorac Surg 1998;66:72
© 1998 The Society of Thoracic Surgeons
Original articles: cardiovascular
Invited commentary
Vincent L. Gott, MDa
a Department of Cardiac Surgery, The Johns Hopkins Hospital, 618 Blalock Building, 600 N Wolfe St, Baltimore, MD 21287-4618, USA
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Invited commentary
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This excellent study from Carpentiers surgical group provides strong evidence that virtually all patients with Marfans syndrome and substantial mitral regurgitation can obtain excellent long-term results with mitral valve repair. Only 1 of the 33 patients required valve replacement, and that patient had severe annular calcification extending into the ventricular myocardium.
Our experience at The Johns Hopkins Hospital supports the conclusions presented by Fuzellier and coauthors. Gillinov and associates [1] reported that 36 mitral valve procedures were performed in 160 consecutive patients with Marfans syndrome having operation at our hospital between January 1983 and January 1993. As opposed to the Hôpital Broussais experience, the majority of these patients (27/36) had concomitant aortic root replacement. Twenty-nine of our 36 patients had valve repair, and none required late mitral valve replacement (only 2 of the 33 patients in the study by Fuzellier and colleagues required late mitral valve replacement).
The histologic finding of altered collagen in mitral leaflets of patients with Marfans syndrome in the Hôpital Broussais experience is in accordance with studies at Johns Hopkins Hospital by Fleischer and coworkers [2]. They found moderate to severe fragmentation of fibrillin in aortic and mitral leaflets of patients with this syndrome. The fact that mitral valve repair in such patients seems to hold up very well even in the presence of major structural protein fragmentation suggests that the aortic valvesparing operation may, in turn, provide much better long-term results than once thought.
Finally, I agree with the Hôpital Broussais group that the long-term prognosis for these patients with Marfans syndrome depends on the aortic complications once the mitral regurgitation has been corrected.
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References
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- Gillinov M., Hulyalkar A., Cameron D., et al. Mitral valve operations in patients with the Marfan syndrome. J Thorac Cardiovasc Surg 1994;107:724-731.[Abstract/Free Full Text]
- Fleischer K.J., Nousari H.C., Anhalt G.J., Stone C.D., Laschinger J.C. Immunohistochemical abnormalities of fibrillin in cardiovascular tissues in Marfans syndrome. Ann Thorac Surg 1997;63:1012-1017.[Abstract/Free Full Text]