CLEFT OF THE MITRAL VALVE IN PATIENTS WITH DOWN'S SYNDROME

Abstract
Differentiation between a cleft of the mitral valve and the cleft of the left side of an atrioventricular septal defect – a lesion commonly found in patients with Down's syndrome - is surgically important since the distribution of the conduction tissue varies between the 2 lesions. In 5 patients with Down's syndrome and cleft of the mitral valve followed in our institution, Echocardiography showed a cleft dividing the anterior (aortic) leaflet of mitral valve with normal papillary muscle position, mural leaflet size, and ratio of the inlet/outlet dimension of the left ventricle. Associated cardiac lesions were present in all 5 patients: perimembranous ventricular septal defect in 3, Ostium secundum atrial septal defect in 2 and patent ductus arteriosus in 2 patients. During the 5.6 years (0.2 to 11) of the follow-up period, surgical repair of the cleft was not indicated since the mitral regurgitation through the cleft remained mild or absent in all the patients. In conclusion, a cleft of the mitral valve has important morphologic differences with atrioventricular septal defect and may occur in patients with Down's syndrome. Functional consequences of the cleft are often minor, although surgical repair may be indicated electively in older patients or if the mitral regurgitation worsen.

Key words
Mitral valve disease, cleft, Down´s syndrome

Bibliografía del artículo

1. Ferencz C, Neill CA, Boughman JA, Rubin JD, Brenner JI, Perry LW. Congenital cardiovascular malformations associated with chromosome abnormalities : an epidemiologic study. J Pediatr 1989 ; 114 : 79-90.
2. Otero Coto E, Quero Jimenez M, Deverall PB, Blain H. Anomalous mitral 'cleft' with abnormal ventriculo-arterial connection: anatomical findings and surgical implications. Pediatr Cardiol 1984 ; 5 : 1-5.
3. Smallhorn JF, de Leval M, Stark J, Sommerville J, Taylor JF, Anderson RH, Macartney FJ. Isolated anterior mitral cleft. Two dimensional echocardiographic assessment and differenciation from « clefts » associated with atrioventricular septal defect. Br Heart J 1982 ; 48 :109-116.
4. Wenink ACG, Gittenberger-de Groot AC, Brom AG. Developmental considerations of mitral valve anomalies. Int J Cardiol 1986 ; 11 : 85-98
5. Sigfùsson G, Ettedgui JA, Silverman NH, Anderson RH. Is a cleft in the anterior leaflet of an otherwise normal mitral valve an atrioventricular canal malformation J Am Coll Cardiol 1995 ; 26 : 508-515.
6. Tamura M, Mehanem S, Brizard C. Clinical feature and management of isolated cleft mitral valve in childhood. J Am Coll Cardiol 2000 ; 35 : 764-770.
7. Kohl T, Silverman NH. Comparison of cleft and papillary muscle position in cleft mitral valve and atrioventricular septal defect. Am J Cardiol 1996 ; 77 : 164-169.
8. Di Segni E Edwards JE. Cleft anterior leaflet of the mitral valve with intact septa. A study of 20 cases. Am J Cardiol 1983 ; 51 : 919-926.
9. Fraisse A, Abdel Massih T, Kreitmann B, Metras D, Vouhé P, Sidi D, Bonnet D. Characteristics and Management of Cleft Mitral Valve. J Am Coll Cardiol 2003 (in press).
10. Fraisse A, Abdel massih T, Vouhé P, Kreitmann B, Gaudart J, Sidi D, Bonnet, D. Management and outcome of patients with abnormal ventriculo-arterial connections and mitral valve cleft. Ann Thorac Surg 2002 ; 74 : 786-91.
11. McDonald RW, Ott GY, Pantely GA. Cleft in the anterior and posterior leaflet of the mitral valve: a rare anomaly. J Am Soc Echocardiogr 1994 ; 7 : 422-424.
12. Perier P, Clausnitzer B. Isolated cleft mitral valve : valve reconstruction techniques. Ann Thor Surg 1995 ; 59 : 56-59.

Autoevaluación