title: Echocardiographic evaluation and surgical implications of common atrioventricular canal defects with absent or diminutive ostium primum defect.
abstract: Common atrioventricular canal defects without ostium primum defects are rare, and their accurate identification has important surgical implications. Retrospective echocardiographic database review identified subjects with common atrioventricular canal defects with absent or diminutive ostium primum defects. Surgical reports and initial and postoperative echocardiograms were reviewed to identify the imaging planes necessary to characterize this anomaly and the surgical challenges imposed by the diagnosis. Fourteen subjects were identified (93% with trisomy 21) with either absent (n = 6) or diminutive (n = 8) ostium primum defects. Malaligned conal septum was present in 50% of subjects with absent primum defects and 12.5% of subjects with diminutive defects. Immediate or long-term complications of the 11 postoperative patients included atrioventricular block (n = 4) and moderate (n = 5) or severe (n = 3) mitral regurgitation. In conclusion, echocardiographic features for the identification of common atrioventricular canal defects with absent or diminutive ostium primum defects are described. Surgical challenges involve attaining adequate exposure of the mitral component and achieving mitral valve competence.
authors.affiliation: Division of Pediatric Cardiology, Mount Sinai School of Medicine, New York, New York, USA.
authors.forename: Avnit
authors.initials: A
authors.lastname: Kaur
authors.forename: Shubhika
authors.initials: S
authors.lastname: Srivastava
authors.forename: Irene D
authors.initials: ID
authors.lastname: Lytrivi
authors.forename: Khanh
authors.initials: K
authors.lastname: Nguyen
authors.forename: Wyman W
authors.initials: WW
authors.lastname: Lai
authors.forename: Ira A
authors.initials: IA
authors.lastname: Parness
mesh_terms: D000015:Abnormalities, Multiple; D006348:Cardiac Surgical Procedures / Q000379:methods*; D003937:Diagnosis, Differential; D004452:Echocardiography / Q000379:methods*; D004694:Endocardial Cushion Defects / Q000000981:diagnostic imaging* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006344:Heart Septal Defects, Atrial / Q000000981:diagnostic imaging* / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D012720:Severity of Illness Index; D016896:Treatment Outcome