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Right-Dominant Unbalanced Atrioventricular Septal Defect: Echocardiography in Surgical Decision Making. Journal of the American Society of Echocardiography Arunamata, A., Balasubramanian, S., Mainwaring, R., Maeda, K., Selamet Tierney, E. S. 2017; 30 (3): 216-226

Abstract

Management of right-dominant atrioventricular septal defect (AVSD) remains a challenge given the spectrum of ventricular hypoplasia. The purpose of this study was to assess whether reported echocardiographic indices and additional measurements were associated with operative strategy in right-dominant AVSD.A blinded observer retrospectively reviewed preoperative echocardiograms of patients who underwent surgery for right-dominant AVSD (January 2000 to July 2013). Ventricular dimensions, atrioventricular valve index (AVVI; left valve area/right valve area), and right ventricular (RV)/left ventricular (RV/LV) inflow angle were measured. A second observer measured a subset of studies to assess agreement. Pearson correlation analysis was performed to examine the relationship between ventricular septal defect size (indexed to body surface area) and RV/LV inflow angle in systole. A separate validation cohort was identified using the same methodology (August 2013 to July 2016).Of 46 patients with right-dominant AVSD (median age, 1 day; range, 0-11 months), overall survival was 76% at 7 years. Twenty-eight patients (61%) underwent single-ventricle palliation and had smaller LV dimensions and volumes, AVVIs (P = .005), and RV/LV inflow angles in systole (P = .007) compared with those who underwent biventricular operations. Three patients undergoing biventricular operations underwent transplantation or died and had lower indexed LV end-diastolic volumes compared with the remaining patients (P = .005). Interobserver agreement for the measured echocardiographic indices was good (intraclass correlation coefficient = 0.70-0.95). Ventricular septal defect size and RV/LV inflow angle in systole had a strong negative correlation (r = -0.7, P < .001). In the validation cohort (n = 12), RV/LV inflow angle in systole = 114° yielded sensitivity of 100% and AVVI = 0.70 yielded sensitivity of 88% for single-ventricle palliation.Mortality remains high among patients with right-dominant AVSD. RV/LV inflow angle in systole and AVVI are reproducible measurements that may be used in conjunction with several echocardiographic parameters to support suitability for a biventricular operation in right-dominant AVSD.

View details for DOI 10.1016/j.echo.2016.10.017

View details for PubMedID 27939051