Spectrum of cardiac lesions associated with Isolated Cleft Mitral Valve and their impact on therapeutic choices

Abstract

BackgroundIsolated cleft mitral valve (ICMV) may occur alone or in association with other congenital heart lesions. The aim of this study was to describe the profile of cardiac lesions associated with ICMV and their potential impact on therapeutic management.MethodsWe conducted a descriptive study with data retrieved from the Congenital Heart Disease (CHD) single-center registry of our institution, including patients with ICMV registered between December 2008 and November 2014.ResultsAmong 2177 patients retrieved from the CHD registry, 22 (1%) had ICMV. Median age at diagnosis was 5 years (6 days to 36 years). Nine patients (40.9%) had Down syndrome. Seventeen patients (77.3%) had associated lesions, including 11 (64.7%) with accessory chordae in the left ventricular outflow tract (LVOT) with no obstruction, 15 (88.2%) had ventricular septal defect (VSD), three had secundum atrial septal defect, and four had patent ductus arteriosus. Thirteen patients (59.1%) required surgical repair. The decision to proceed with surgery was mainly based on the severity of the associated lesion in eight patients (61.5%) and on the severity of the mitral regurgitation in four patients (30.8%). In one patient, surgery was decided based on the severity of both the associated lesion and mitral regurgitation.ConclusionOur study shows that ICMV is rare and strongly associated with Down syndrome. The most common associated cardiac abnormalities were VSD and accessory chordae in the LVOT. We conclude that cardiac lesions associated with ICMV are of major interest, since in this study patients with cardiac lesions were diagnosed earlier. The decision to operate on these patients must take into account the severity of both mitral regurgitation and associated cardiac lesions.

Diagnosis of Isolated Cleft of the Anterior Mitral Leaflet in a Dog: A Case Study Using Real-Time Three-Dimensional Echocardiography

“…Although anterior MVC can be encountered within the spectrum of atrioventricular septal defects (AVSD), it is rare for them to be diagnosed as an isolated finding in dogs. ICAML is assumed to be a partial defect of the endocardial bed [ 1 – 3 ]. In human medicine, the median duration from birth to diagnosis is reported to be 2 or 5 years [ 1 , 3 ].…”

“…Isolated cleft of the anterior mitral leaflet (ICAML, not associated with atrioventricular septal defect) is a very rare cause of congenital mitral regurgitation (MR) in humans [ 1 – 3 ]. Only one case has been reported on the occurrence of ICAML in dogs [ 4 ].…”

Co‐occurring medical conditions in adults with Down syndrome: A systematic review toward the development of health care guidelines. Part II

“…In children with DS, CVD is associated with CHD such as atrioventricular septal defect, ventricular septal defect and Tetralogy of Fallot (Acar et al 1999; Tumanyan et al 2015). Isolated cleft mitral valve can also occur in DS even in the absence of CHD (Hammiri et al 2016), and its prevalence in DS may be around 6% (Thankavel and Ramaciotti 2016).…”

Diagnosis of mitral valve cleft using real-time 3-dimensional echocardiography

“…The 3D full-volume imaging mode does not collect images from the same cardiac cycle. Therefore, errors in imaging consistency may occur when the patient changes his or her position, takes deep breaths or has cardiac arrhythmia (26,27). Furthermore, the surface area of the volume transducer is large enough to affect the complete display of the entire structure due to the narrow intercostal space, which can limit the echo-wave through the observed window (28).…”