Asian Cardiovasc Thorac Ann 2005;13:181-183
© 2005 Asia Publishing EXchange Ltd
Mitral Valve Repair and Cryo-Maze Procedure in Ehlers-Danlos Syndrome
Tomoyuki Goto, MD,
Junjiro Kobayashi, MD,
Hiroyuki Nakajima, MD,
Kazuo Niwaya, MD,
Osamu Tagusari, MD,
Soichiro Kitamura, MD
Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan
For reprint information contact: Junjiro Kobayashi, MD Tel: 81 6 6833 5012 Fax: 81 6 6872 7486 Email: jkobayas{at}hsp.ncvc.go.jp, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565–8565, Japan.
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ABSTRACT
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A 48-year-old man presented with typical features of Ehlers-Danlos syndrome, such as soft and hyperextensible skin, subcutaneous nodules, clubbed feet, and a mild degree of pigeon chest. In addition, his past history revealed congenital dislocation of the hip joint, retinal detachment, and repeated episodes of congestive heart failure due to severe mitral regurgitation and atrial fibrillation. We subjected the patient to surgical repair of the mitral valve combined with the maze procedure, which proved successful.
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INTRODUCTION
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The Maze procedure for atrial fibrillation has been applied successfully in patients with various structural heart diseases. Ehlers-Danlos syndrome (EDS) is a connective tissue disorder characterized by hyperextensibility of the skin, fragility of large and small vessels, hypermobility of joints and poor wound healing with atrophic scarring.1 A successfully performed Maze procedure and avoidance of mechanical valves allows patients to lead an active life without the need for anticoagulation therapy. This is especially true for patients with EDS because such patients have an aortic lesion and tend to bleed easily due to the vulnerability of the vessels.2
We herein report the case of a patient with EDS in whom we successfully performed a surgical repair of the mitral valve in combination with the Maze procedure.
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CASE REPORT
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A 48-year-old man with EDS was referred to our institution for treatment of mitral regurgitation and atrial fibrillation. He had been diagnosed with EDS, presenting with hyperextensible skin, subcutaneous nodules, clubbed feet, and a mild degree of pigeon chest. His past history revealed an episode of congenital dislocation of the hip joint and detachment of the retina without obvious family history.
Atrial fibrillation had been experienced for 10 months and was managed with digoxin. A chest radiography obtained on admission showed a cardiothoracic ratio of 0.65. Echocardiography demonstrated grade 4 mitral regurgitation due to posterior leaflet prolapse (Figure 1
). The left atrial dimension increased to 68 mm in systole. Cardiac catheterization revealed that left ventricular end-diastolic volume index and ejection fraction were 149 mL·m–2 and 48%, respectively.
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Figure 1. Echocardiography demonstrated grade 4 mitral regurgitation due to posterior leaflet prolapse. (A) Long axis view. (B) Short axis view.
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At surgery, we approached the mitral valve via a right-sided left atriotomy. We performed cryo-Maze procedure including cryo-ablation for pulmonary venous isolation from the left atrium. The mitral valve was inspected. The findings included a remarkably dilated annulus and a torn chorda of the middle scallop of the posterior leaflet with dysplasia of the posteromedial commissural scallop (Figure 2). The valve was repaired with quadrangular resection of the posterior leaflet and annuloplasty using a 27 mm Duran ring (Medtronic, Minneapolis, MN, USA). Pathological examination revealed that the fibrous tissue of the posterior mitral leaflet had partially vanished and diminished, and that there was severe myxomatous degeneration of the posterior mitral leaflet with deposit of proteoglycans.
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Figure 2. From the surgeon s view, remarkably dilated annulus and torn chorda of the middle scallop of the posterior leaflet with dysplasia of the posteromedial commissural scallop were seen.
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Postoperative echocardiography revealed mild mitral regurgitation. However, no progression of mitral regurgitation was observed at 1 year follow-up. Sinus rhythm was maintained successfully without anti-arrhythmic drugs and the use of a mechanical valve and anticoagulation therapy was avoided.
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DISCUSSION
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EDS is a connective tissue disorder characterized by hyperextensibility of the skin, joint hypermobility, fragility of the blood vessels, and increased susceptibility to bleeding. It was reported that type IV EDS results from mutations in the gene for type III procollagen,1 however, the molecular defect of the common type of EDS has not been identified as yet.3 Cardiovascular abnormalities are also typical manifestations of EDS and may affect the life span.2
In mitral regurgitation reconstruction of the valve is considered the procedure of choice, even for patients with an underlying progressive connective tissue disorder. In Marfan’s syndrome, greater understanding of the complicated anatomy of valvular and subvalvular structures has improved the quality of mitral valve repair. It has been widely accepted that the early and intermediate-term durability in Marfan’s syndrome was similar to that of non-Marfan degenerative mitral valve disease. Fuzellier and colleagues reported that freedom from mitral valve re-operation was 87.1% at 10 years,4 while Gillinov and coworkers reported that the actuarial freedom from significant regurgitation was 88.3% at 5 years.5 In patients with EDS, no detailed anatomical characteristics of valve structures peculiar to this syndrome have been delineated as yet. Since there are only a few reports of mitral valve surgery in patients with EDS, the durability of the repaired mitral valve remains unclear.6,7
In the present case, mitral regurgitation was due to a torn chorda with severe myxomatous degeneration. Regurgitation could be effectively reduced by quadrangular resection of the prolapsing part of the posterior leaflet. The pathological examination of the surgical specimen revealed disruption of the fibrous structure in the leaflet and chordal tissue similar to that seen in patients with degenerative mitral regurgitation.4 Elimination of atrial fibrillation is also essential to avoid anticoagulation therapy. To facilitate the recovery of sinus rhythm, we performed a modified Maze procedure. Our current modified Maze procedure, previously reported in detail,8 consists of pulmonary venous isolation and interruption of atrial conduction using cryo-ablation through a standard right-sided left atriotomy. The cryo-Maze procedure requires less than 30 minutes of additional aortic cross-clamp time, and the risk of perioperative bleeding is considered minimal. After the Maze procedure, warfarin is routinely administered to all patients for 3 months. If sinus rhythm is constantly maintained, anticoagulation therapy is terminated in patients with mitral valve repair or biological valve implantation. Small dosages of aspirin are given if the contraction of the left atrium is absent or left atrial dimension is greater than 55 mm.
In conclusion, mitral valve repair concomitant with the Maze procedure may be valuable and rationalized in order to decrease the late complications associated with anticoagulation, and thus improve the quality of life of patients with EDS.
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REFERENCES
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- Pepin M, Schwarze U, Superti-Furga A, Byers PH. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med
2000;342:673–80.[Abstract/Free Full Text]
- Mattar SG, Kumar AG, Lumsden AB. Vascular complications in Ehlers-Danlos syndrome. Am Surg
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- Byers PH. Ehlers-Danlos syndrome: recent advances and current understanding of the clinical and genetic heterogeneity. J Invest Dermatol
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- Fuzellier JG, Chauvaud SM, Fornes P, Berrebi AJ, Lajos PS, Bruneval P, et al. Surgical management of mitral regurgitation associated with Marfan’s syndrome. Ann Thorac Surg
1998;66:68–72.[Abstract/Free Full Text]
- Gillinov AM, Hulyalkar A, Cameron DE, Cho PW, Greene PS, Reitz BA, et al. Mitral valve operation in patients with the Marfan syndrome. J Thorac Cardiovasc Surg
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- Kitagawa M, Nakagawa Y, Shibairi M, Yasuno K, Okimoto M, Masuda M, et al. Mitral valve replacement in a case of the Ehlers-Danlos syndrome. Nippon Kyobu Geka Gakkai Zasshi
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- Nakajima H, Kobayashi J, Bando K, Niwaya K, Tagusari O, Sasako Y, et al. The effect of cryo-maze procedure on early and intermediate term outcome in mitral valve disease: case matched study. Circulation
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