부산대학교 도서관

Pathologies of the left ventricular outflow tract and of the ascending aorta are relatively rare congenital lesions. We assessed the early outcome and long-term follow-up of neonates, children and adolescent patients who were treated surgically because of a pathology of the left ventricular outflow tract (subaortic stenosis and/or valvular aortic stenosis) or of the ascending aorta.APaBetween 1984 and 1999, 96 patients were treated in our institution: 68 patients underwent a primary operative procedure, 22 had a redo-intervention whereas in 6 patients a re-re-intervention was performed. Primary isolated valvotomy was performed in 30 neonates or young children below 6 months. In the group requiring redo-surgery, the following procedures were performed: repeated valvotomy with or without reconstruction of the valve by commissural plication or cusp extension (n=8), valve replacement with homograft (n=10), with mechanical (n= 7) and biological prosthesis (n=3). In patients with a subaortic obstruction, a membranous stenosis was resected in 10, a muscular resection was performed in 5 and in 3 patients a more complex procedure (Ross-Konno) was performed.APaThe following surgical procedures were performed in those patients presenting with a pathology of the aortic root and/or of the ascending aorta: aortic root replacement with preservation of the native valve (n=5), composite graft replacement (n=4) and homograft mini-root (n=2), whereas in 4 patients with idiopathic dilatation of the ascending aorta, a supracoronary graft was implanted. In 3 patients presenting with a supravalvar aortic stenosis and Williams-Beuren syndrome, a xenopericardial patch was inserted in 2 and a graft was implanted in one.APaOverall mortality was 4% (3/96) interestingly there was no mortality following redo-procedures. The following significant complications were observed in neonates following aortic valvotomy: low cardiac output (n=6/30, 20%), av block III (1/30, 3%), renal failure requiring peritoneal dialysis (4/30, 16%). Predictive factors for perioperative mortality in this group were: age