Congenital Anomalies of the Mitral Valve

Case Report: Double Orifice Mitral Valve with Cleft in Anterior Leaflet of Dominant Valve in an Afro-Caribbean

“…The MV abnormality is believed to be secondary to genetic mutations affecting the regulatory proteins in the myocytes. 8 A familial case involving a brother and sister has been described, indicating an underlying chromosomal or genetic cause; 11 however, the index case had no family history of congenital heart disease or MV disease. 85% of DOMV patients have a dominant orifice and smaller vestigial one as in the index case.…”

“…14 All the aforementioned symptoms and signs are well documented. [3][4][5][6][7][8][9][10][11][12][13][14] Hence the management of DOMV is based on the patient's individual anatomy and physiology. This is usually only needed when there is significant MR and MS. 3,14 This case confirms that complex structural cardiac anatomy can be clearly defined in expert hands.…”

“…The list of associated conditions include: atrioventricular septal defect, primum atrial septal defect, ventricular septal defect, atrial septal defect, truncus arteriosus, coarctation of the aorta, interruption of the aortic arch, tetralogy of Fallot, corrected transposition, bicuspid aortic valve, pulmonary stenosis, Ebstein's anomaly of the tricuspid valve, dysplastic tricuspid valve, double orifice tricuspid valve, tricuspid atresia, parachute mitral valve, marfanoid features, left ventricular noncompaction, atrial tachycardia, congenital complete heart block, and cardiomyopathy. [1][2][3][4][5][6][7][8][9][10][11][12][13][14] Presentation may not occur, hence being totally asymptomatic with competent DOMV. 4 Mitral regurgitation (MR) occurs in the majority of cases (45%) and mitral stenosis (MS) in 11%, in either or both MVs to varying degrees of severity.…”

“…There are varied abnormalities of the MV annulus, the tensor apparatus and papillary muscles which may be duplicated, with each MV having its own complete subvalvular attachments to the left ventricle (LV). [1][2][3][4][5][6][7][8] DOMV may occur with or without noncompaction of the LV. 5 They are then classified into three main groups anatomically by Trowitzsch et al 2 as Type 1, having a complete bridge going between the valves as in the index case, Type 2, an incomplete bridge with connections at the edge of the leaflets of the MVs, and Type 3, a hole in the lateral commissure.…”