AORTIC CUSP PROLAPSE IN VENTRICULAR SEPTAL-DEFECT AND ITS ASSOCIATIONWITH AORTIC REGURGITATION - APPROPRIATE TIMING OF SURGICAL REPAIR ANDOUTCOMES

OBJECTIVES: To determine the appropriate indications and timing for su rgery in children with either a perimembranous or a subarterial type o f ventricular septal defect (VSD) associated with aortic cusp prolapse . DESIGN: Retrospective review of children with VSD and associated aor tic cusp prolapse with or without aortic regurgitation. This review wa s based on data obtained from clinical findings, two-dimensional echoc ardiography, cardiac catheterization and angiocardiography. SETTING: T ertiary health care facility with two-dimensional and colour Doppler e chocardiographic and cardiac surgery facilities, and a catheterization laboratory. PATIENTS: Forty-eight patients were found to have perimem branous or subarterial VSDs in association with aortic cusp prolapse w ith or without aortic regurgitation. INTERVENTIONS: All 48 patients ha d high resolution two dimensional and colour Doppler echocardiography. Of the 19 patients who underwent surgical closure of their VSD, five also had an aortic valvuloplasty and one had an aortic valve replaceme nt. Cardiac catheterization was performed in 16 of the 19 surgical pat ients and 12 of the 29 nonsurgical patients. MEASUREMENTS AND MAIN RES ULTS: Annual clinical and echocardiographic assessments in the nonsurg ical group did not demonstrate increasing aortic insufficiency. Two ch ildren in the nonsurgical group showed spontaneous resolution of aorti c in sufficiency. In the surgical group, four children with VSD and cl inical aortic insufficiency had surgery at less than five years of age ; two were found to be regurgitant-free, one had trivial clinical aort ic insufficiency and the other had echocardiography-only insufficiency . Of the seven surgical patients older than five years with VSD and cl inical aortic insufficiency, four were found to be regurgitant-free, o ne had echocardiography-only regurgitation and two were unchanged. Two children undergoing surgery with VSD and Ilo aortic insufficiency had postoperative echocardiography-only regurgitation, presumably related to cusp deformity from presurgical prolapse. Children with large VSDs with or without aortic cusp prolapse required surgery for indications of shunt size and pulmonary resistance. CONCLUSIONS: For children wit h small perimembranous VSDs and cusp prolapse, surgery is indicated on ly if there is clinical evidence of aortic regurgitation and progressi ve left ventricular enlargement.