Abstract 13174: Coronary Origin Patterns: Utility in Echocardiographic Diagnosis of Forme Fruste Right-left Bicuspid Aortic Valve

Abstract

Introduction: Bicuspid aortic valve (BAV) is a common congenital heart defect associated with coronary artery (CA) variants, including higher incidence of left CA dominance and shorter left main CA length. In patients with right-left fusion (R/L) BAV, we observed that the origins of left and right coronaries are in closer proximity than in normal patients. ‘Forme fruste’ types of R/L BAV are subtle and difficult to diagnose, hence this coronary pattern can aid in diagnosis. We defined intercoronary distance as the angle separating the left and right coronary ostia relative to the aortic valve center. Hypothesis: The coronary angle is more acute in R/L BAV than in normal aortic valves. Methods: A retrospective review of pediatric echocardiograms with R/L BAV at a single institution (12/2010-11/2018) was performed; coronary angles were measured and compared to age-matched controls. Those with inadequate images or anomalous coronary origins were excluded. Results: We identified 193 patients with R/L BAV (64% male) and 136 patients with normal aortic valves (57% male). Coronary angle was significantly more acute in R/L BAV than in controls (117.7°±16.8° vs 139.0°±10.1°, p<0.0001). This was independent of age, gender, or other cardiac defects. This relationship remained when BAV patients with abnormal aortic Z-scores were removed from analysis (119.2°±15.3° vs 139.0°±10.1°, p<0.0001), ruling out an effect of abnormal aortic annulus or root. Conclusions: Coronary origins are in closer proximity in R/L BAV independent of annulus/root geometry, associated cardiac defects, age, or gender. This new finding may assist in echocardiographic diagnosis of subtle subtypes of BAV.