Transesophageal echocardiographic guidance for surgical repair of aortic insufficiency in congenital heart disease.

A total of 14 patients with congenital heart disease underwent aortic valve repair, with transesophageal echocardiograms performed to determine severity, mechanism, and direction of aortic insufficiency (AI) jet to tailor the surgical approach. Patient age was 13 +/- 10 years, and accompanying diagnoses were: truncus arteriosus, subaortic stenosis, ventricular septal defect, and tetralogy of Fallot. Repeat transesophageal echocardiography was performed after each cardiopulmonary bypass run to determine residual AI and mechanism. Aortic valve leaflet number ranged from 2 to 4. AI was graded 2 to 4+, and postoperatively 0 to 2+. Primary mechanisms were: cusp prolapse (7), leaflet holes (4), restricted leaflet motion (2), and annular dilation (3). Patients required 1 to 3 cardiopulmonary bypass runs until primary AI mechanism was abolished. In all, 12 of 14 patients were free from death or repeated surgery at 2 years. Surgical repair of congenital AI may be aided by transesophageal echocardiographic guidance, with repeat short cardiopulmonary bypass as needed. Long-term studies are needed to determine durability of repair.