title: Severe Left Atrioventricular Valve Regurgitation Due to Discontinuity between the Leaflets of the Aortic and Left Atrioventricular Valves in a Patient with Endocardial Cushion Defect: A Rare Case Report.
abstract: Discontinuities between the leaflets of the aortic and left atrioventricular valves are uncommon congenital malformations. The anomaly may be discovered during surgery without preoperative diagnosis. It represents a spectrum of anomalies that result from interruption of the normal development of the endocardial cushions during the fetal life. We describe a rare case of Down syndrome with transient complete atrioventricular block and discontinuity between the leaflets of the aortic and left atrioventricular valves without intervening fibrous band, leading to separation and detachment between them. It caused severe eccentric jet of regurgitation originated from left ventricular outflow tract and base of anterior leaflet of left atrioventricular valve into the left atrium. He underwent cardiopulmonary bypass, and the defect between left atrioventricular valve and aortic annuli was sewn. Permanent epicardial pacing was inserted during cardiac surgery. To the best of our knowledge, such a case has not been previously reported in the literature.
authors.affiliation: Fellowship of Echocardiography, Department of Cardiology, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, Iran.
authors.forename: Maryam
authors.initials: M
authors.lastname: Nabati
authors.affiliation: Department of Cardiac Surgery, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, Iran.
authors.forename: Valiolla
authors.initials: V
authors.lastname: Habibi
authors.affiliation: Department of Cardic Anesthesiology, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, Iran.
authors.forename: Aria
authors.initials: A
authors.lastname: Soleimani
authors.affiliation: Mazandaran University of Medical Sciences, Sari, Iran.
authors.forename: Mojtaba
authors.initials: M
authors.lastname: Shokri
mesh_terms: D000293:Adolescent; D001022:Aortic Valve Insufficiency / Q000000981:diagnostic imaging* / Q000209:etiology*; D003937:Diagnosis, Differential; D004452:Echocardiography / Q000379:methods*; D004694:Endocardial Cushion Defects / Q000150:complications* / Q000000981:diagnostic imaging*; D006351:Heart Valves / Q000002:abnormalities* / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D035583:Rare Diseases / Q000000981:diagnostic imaging
keywords: endocardial cushion defect; heart block; pacemaker; surgery