Surgical management of ventricular septal defect with aortic valve prolapse: clinical considerations and results

Aortic valve prolapse is found in over 5% of children with ventricularseptal defect (VSD). Although this association occurs mostly with doublycommitted subarterial VSDs, in this study the predominant type of VSD wasperimembranous. In order to determine the need and timing for surgery andwhether the anatomical features of septal defect may influence clinicalmanagement and outcome in this lesion, we reviewed our experience with 28consecutive patients, operated on for VSD with prolapsed aortic valve cusp, with or without aortic regurgitation. Twenty-two patients had aperimembranous VSD and six had doubly committed VSD. Aortic regurgitationwas trivial or absent in nine patients, mild in ten and moderate to severein nine. Associated cardiac anomalies were present in 18 patients, allhaving perimembranous VSD, and included right ventricular outflow tract(RVOT) obstruction (n = 6), discrete subaortic membrane (n = 4) or both (n= 8). None of these patients had more than moderate aortic regurgitation.The patients underwent surgical closure of the septal defect between theages of 1.5 and 34 years of age (median = 7). Sixteen patients having mildor trivial aortic regurgitation underwent closure of the VSD only, and 12patients underwent VSD closure with aortic valvuloplasty. Valvuloplasty wasrequired more often in doubly committed VSDs (66%) and in theperimembranous type without associated anomalies (100%), and significantlyless often in the presence of RVOT obstruction, subaortic membrane or both(22%). At follow-up (up to 5 years, mean 18 months), the grade of aorticregurgitation was unchanged in 11 and decreased in 5 patients undergoingclosure of the VSD only.