Unbalanced atrioventricular septal defect: defining the limits of biventricular repair

Unbalanced atrioventricular septal defect (uAVSD) is a challenging lesion with suboptimal outcomes in the current era. Severe forms of uAVSD mandate univentricular repair with well-documented outcomes. Determining the feasibility of biventricular repair (BVR) in patients with moderate forms of uAVSD is difficult. Ventricular hypoplasia has traditionally formed the cornerstone of defining uAVSD. However, malalignment of the atrioventricular junction and related derangements of the anatomy and physiology of the atrioventricular inflow play a central role in establishing and sustaining a biventricular end state. Atrioventricular valve index, left ventricular inflow index, and right ventricle/left ventricle inflow angle are important recently described measures of inflow physiology. Additional patient anatomic and physiologic factors that impact BVR feasibility undoubtedly exist. A recently launched Congenital Heart Surgeons Society prospective inception cohort study will address these and other issues that impair our ability to predict BVR feasibility in uAVSD.