Coronary arterial anatomy in double-outlet right ventricle with subpulmonary VSD

We have examined 38 hearts with a double-outlet right ventricle with a subpulmonary ventricular septal defect. We divided the hearts into three groups according to the angle between the planes formed between the outlet septum and the remainder of the muscular ventricular septum; namely, at approximately right angles (15 hearts), parallel (11 hearts), and at an acute angle (12 hearts). The coronary arterial pattern corresponding to that seen in the normal heart was present in 11 hearts (73%) of the "right angle" group, in only one heart (8%) of the "acute angle" group, and in none of the "parallel" group. In contrast, the most common pattern in the setting of complete transposition was observed in none, 8%, and 91% of each group, respectively. Other diverse patterns were recognized in the hearts in the acute angle group, and the incidence of abnormal branching was significantly higher in this than in the other groups (p < 0.01). Knowledge of these anatomic variations in the course of the coronary arteries, some of which would cause problems at either definitive repair or reoperation, are essential for those seeking to achieve optimal surgical repair.