Isolated anterior mitral cleft. Two dimensional echocardiographic assessment and differentiation from "clefts" associated with atrioventricular septal defect.

Abstract

SUMMARY Five patients with isolated clefts in the anterior leaflet of the mitral valve, unassociated with atrioventricular septal defects, are described. All had significant mitral regurgitation, with the cleft being the only abnormality in three. Two patients had an associated ventricular septal defect, one with a straddling right atrioventricular valve. Angiocardiography in four showed moderate regurgitation, but was not able to delineate the aetiology. Two dimensional echocardiography showed a constant defect in the anterior leaflet, pointing towards the left ventricular outflow tract. This differed from 30 cases with atrioventricular septal defects where the "cleft" pointed towards the interventricular septum and was situated between the anterior and posterior bridging leaflets. All cases with isolated clefts had surgical correction, with minimal residual regurgitation on follow-up examination in two cases. Our current policy in patients with uncomplicated isolated cleft involves non-invasive assessment of these children and surgical correction if the regurgitation is significant. Most

Characteristics and management of cleft mitral valve

“…In our patients, the cleft was clearly in a more anterior direction toward the aortic root (Fig. 1), as it was noticed in previous echocardiographic and postmortem studies (3,5,6,8,11), although this position may vary according to precise echocardiographic measurements performed by Khol and Silverman (7).…”

“…The age at presentation in our study patients ranged from 0 to 10.6 years, related in some cases to the various functional consequences of the associated cardiac lesions and to extracardiac genetic features. As in previously reported studies (3,6,8,11), MV repair was feasible in all patients and was indicated in children with symptoms and/or moderate MR by echocardiography. A direct and complete suture of the cleft was accomplished in the majority.…”

“…The papillary muscles are positioned laterally with a smaller mural leaflet (5)(6)(7)9). The cleft points toward the inlet septum (3,4,8). In our patients, the cleft was clearly in a more anterior direction toward the aortic root (Fig.…”

“…A relatively uncommon lesion, such as a cleft of the mitral valve (MV), is morphologically different from a cleft of the left-sided valve of an atrioventricular (AV) septal defect because of the absence of a common AV junction (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11). It is observed when there is mitral regurgitation (MR), subaortic stenosis, or associated lesions (1-3,6 -8).…”

Familial atrioventricular septal defect: possible genetic mechanism.

“…3 The high frequency of isolated mitral cleft in families with atrioventricular septal defects,45 including our family, and its prevalence in patients with Down's syndrome (4/420 v 3/5200 in our experience), however, suggests that this malformation should be included in the spectrum of atrioventricular septal defects.…”