Doubly committed ventricular septal defect (VSD) with ventricular dilatation

Background: The morphology of ventricular septal defects (VSDs) that are doubly committed and juxtaarterial places the patient at risk for aortic valvar prolapse and aortic valvar insufficiency (AI). Surgical repair of this type of defect often involves placing sutures through the base of one or more of the leaflets of the pulmonary valve, raising concern for late pulmonary valvar insufficiency (PI). The purpose of this review was to analyze the postoperative follow-up relating to potential late complications with the aortic and pulmonary valves.

Methods: Between 1980 and 2012, 106 patients with doubly committed juxtaarterial VSD underwent intracardiac repair. Median age at repair was 1.1 years. Preoperative evaluation showed 69 patients (65%) had aortic valvar prolapse and 51 (48%) had AI. Operative approach was through the pulmonary trunk in 88 (83%) of the patients. In 81 patients (76%), sutures securing the VSD patch had been placed through the base of the pulmonary valvar leaflets.

Results: Operative survival was 100%. Follow-up ranges from 6 months to 17 years, with a mean of 4.9 years. No patient had heart block or residual shunting. Of the 70 patients with long-term contemporary echocardiographic follow-up, 66 (94%) had trivial or no AI and 4 (6%) had mild AI. Of these patients, 49 (70%) had trivial or no PI, and 21 (30%) had mild PI. In 1 patient having aortic valvoplasty at the time of VSD closure, the aortic valve was replaced 7 months later. No other patient had worrisome progression of their AI or PI.

Conclusions: The incidence of aortic valvar prolapse and AI in the setting of doubly committed juxtaarterial VSD is quite high. The optimal surgical approach is through the pulmonary trunk. Sutures placed through the base of the pulmonary valvar leaflets do not predispose to clinically significant late pulmonary valvar insufficiency. Timely surgical closure of this type of defect prevents progression of AI.