Total amount: € 0,00
Indexed/Abstracted in: EMBASE, Scopus, Emerging Sources Citation Index
Online ISSN 1827-1782
Aoyagi S., Fukunaga S., Tomoeda H., Ueda T.
Department of Surgery, Kurume University School of Medicine, Kurume, Japan
We report a very rare congenital anomaly consisting of an anomalous fibrous band connecting the anterior mitral leaflet (AML) to the posterior mitral leaflet (PML) in a patient with Noonan syndrome. A 29-year-old man, who had a history of Noonan syndrome, spontaneously closed ventricular septal defect, and hypertrophic cardiomyopathy, presented with mitral regurgitation (MR) and persistent atrial fibrillation. Echocardiography showed an anomalous fibrous band connecting the AML to the PML and moderately thickened, redundant mitral valve leaflets with severe MR resulting from torn chordae tendineae of the AML. No other cardiac abnormalities were detected. At operation, segmental prolapse of the AML and an anomalous fibrous band running from the clear zone, not the leaflet edge, of the AML to that of the PML were confirmed. The mitral valve orifice was normal and no accessory orifice was present. No chordae tendineae were attached to the fibrous band. Histological examination demonstrated that the fibrous band was composed of fibrous connective tissue. The anomalous fibrous band must be congenital, and an abnormal development of the endocardial cushions may be involved into the pathogenesis of this anomaly.