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J Thorac Cardiovasc Surg 2006;131:1400-1401
© 2006 The American Association for Thoracic Surgery
Brief Communication |
Department of Cardiothoracic Surgery and Anaesthesia, Karolinska University Hospital, Stockholm, Sweden.
Received for publication January 19, 2006; revisions received February 2, 2006; accepted for publication February 7, 2006. * Address for reprints: Anders Franco-Cereceda, MD, PhD, Department of Cardiothoracic Surgery and Anaesthesia, Karolinska University Hospital, S-171 76 Stockholm, Sweden. (Email: andfra{at}ki.se).
Here we present the outcome of surgical treatment of 2 identical twins with mitral valve regurgitation (MR) and left ventricular (LV) dilatation.
Clinical Summary
A 52-year-old asymptomatic man (patient 1) presented with a newly detected cardiac murmur at a routine check-up. Further evaluation revealed a large (III-IV/IV) MR and LV dilatation (LV end-diastolic diameter [LVEDD] and LV end-systolic diameter [LVESD] of 62 and 38 mm, respectively). The LV function was preserved, with en ejection fraction (EF) of 60%. During cardiopulmonary bypass, a large posterior leaflet prolapse with chordal rupture was noted. A quadrangular resection of the P2 segment was performed combined with a sliding plasty and application of a 34-mm Carpentier-Edwards anuloplasty ring. The postoperative course was uneventful, and at 12 months' follow-up, there was a trace (0-I/IV) of MR, the LVEDD was reduced to 50 mm, the LVESD was reduced to 36 mm, and the EF was maintained (55%; Figure 1).
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in combination with mitral valve surgery. Ethical permission was then obtained from the hospital ethics committee, and written consent was obtained from the patient. The CSD is a mesh-like polyester fabric with bidirectional compliance placed around the heart to reduce wall stress and reshape the dilated heart from a spherical to a more ellipsoidal shape.
1
The operation was performed with a quadrangular resection of the P2 segment, and a 34-mm Carpentier-Edwards anuloplasty ring was applied because of a large posterior leaflet prolapse and chordal rupture. Initially, a 32-mm ring was applied but changed to a 34-mm ring combined with an edge-to-edge stitch to avoid systolic anterior motion of the mitral valve. After an uneventful recovery, the patient was evaluated at 12 months postoperatively, revealing a minimal MR (0-I/IV), a normalized heart size (LVEDD, 52 mm; LVESD, 44 mm) and an EF of 50% (Figure 1).
Discussion
One of the most common causes of MR necessitating surgical intervention is primary chordal rupture.
2
The cause of the chordal rupture is usually a myxomatous mitral valve in which the mechanical properties of the chordae are affected.
There are several indications of a genetic link to myxomatous mitral valve disease. Thus patients with autosomal dominant polycystic kidney disease have an increased occurrence of cardiac valve abnormalities, and in patients with Marfan syndrome caused by fibrillin gene defects, myxomatous mitral valve disease is the leading cause of mitral regurgitation. Moreover, a high frequency of the angiotensin-converting enzyme II genotype has been demonstrated in myxomatous mitral valve disease, and a gene associated with the disease has been located on Xq28.
3
The presented patients had remarkably similar morphologic findings, with a large myxomatous valve and posterior leaflet prolapse combined with chordal rupture. Both of the patients, in addition, had an enlargement of the left ventricle. Eliminating the MR, if performed early, will reduce the volume overload, allowing the left ventricle to recover in size and function. The timing of surgical intervention in asymptomatic patients might be difficult, but when the EF decreases to 60% or less, the LVESD increases to greater than 40 mm, or both, surgical intervention is advocated.
4
Patient 2 came to surgical intervention almost a year later than patient 1 and was found to have a more severely enlarged heart. In view of the importance of promoting ventricular reversed remodeling and ensuring a reduction of ventricular size to minimize the risk of potential residual functional MR, the CSD was positioned around the heart. Obviously this proved to be most efficient because the heart rapidly decreased in size with maintained LV function. To what extent passive containment operations should be applied more generally in patients with cardiac LV dilatation remains to be established.
Footnotes
Supported by the Mats Kleberg Foundation.
References
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