Umbalanced AV septal defect: Surgical decision making based on echo

Circulation. 2010;122:S209-S215

Echocardiographic Definition and Surgical Decision-Making in Unbalanced Atrioventricular Septal Defect
A Congenital Heart Surgeons’ Society Multiinstitutional Study

Anusha Jegatheeswaran, MD; Christian Pizarro, MD; Christopher A. Caldarone, MD; Meryl S. Cohen, MD; Jeanne M. Baffa, MD; David B. Gremmels, MD; Luc Mertens, MD, PhD; Victor O. Morell, MD; William G. Williams, MD; Eugene H. Blackstone, MD; Brian W. McCrindle, MD, MPH; David M. Overman, MD

Background—Although identification of unbalanced atrioventricular septal defect (AVSD) is obvious when extreme, exact criteria to define the limits of unbalanced are not available. We sought to validate an atrioventricular valve index (AVVI) (left atrioventricular valve area/total atrioventricular valve area, centimeters squared) as a discriminator of balanced and unbalanced forms of complete AVSD and to characterize the association of AVVI with surgical strategies and outcomes.

Methods and Results—Diagnostic echocardiograms and hospital records of 356 infants with complete AVSD at 4 Congenital Heart Surgeons’ Society (CHSS) institutions (2000–2006) were reviewed and AVVI measured (n=315). Patients were classified as unbalanced if AVVI0.4 (right dominant) or 0.6 (left dominant). Surgical strategy and outcomes were examined across the range of AVVI. Competing risks analysis until the time of commitment to a surgical strategy examined 4 end states: biventricular repair (BVR), univentricular repair (UVR), pulmonary artery banding (PAB), and death before surgery. A prediction nomogram for surgical strategy based on AVVI was developed.
The majority of patients had balanced AVSD (avvi between 0.4 and 0.6)
Conclusions—AVVI effectively characterizes the transition between balanced and unbalanced AVSD with important correlation to anatomic substrate and selected surgical strategy.