Van Arsdell G S, Williams W G, Boutin C, Trusler G A, Coles J G, Rebeyka I M, Freedom R M
Division of Cardiovascular Surgery, Hospital for Sick Children, Toronto, Ontario, Canada.
J Thorac Cardiovasc Surg. 1995 Nov;110(5):1534-41; discussion 1541-2. doi: 10.1016/S0022-5223(95)70077-3.
From July 1982 through September 1994, 19 children had operative treatment of subaortic stenosis associated with an atrioventricular septal defect. Specific diagnosis were septum primum defects in 7, Rastelli type A defects in 6, transitional defects in 4, inlet ventricular septal defect with malattached chordae in 1, and tetralogy of Fallot with Rastelli type C defect in 1. Twenty-seven operations for subaortic stenosis were performed. Surgical treatment of the outlet lesion was performed at initial atrioventricular septal defect repair in 3 children and in the remaining 16 from 1.2 to 13.1 years (mean 4.9 years, median 3.9 years) after repair. Eighteen of the 19 children had fibrous resection and myectomy for relief of obstruction. Seven children had an associated left atrioventricular valve procedure. One child received an apicoaortic conduit. Seven children (36.8%) required 8 reoperations for previously treated subaortic stenosis. Time to the second procedure was 2.8 to 7.4 years (mean 4.9 years). Follow-up is 0.4 to 14.0 years (median 5.6 years). Six-year actuarial freedom from reoperation is 66% +/- 15%. The angle between the plane of the outlet septum and the plane of the septal crest was measured in 10 normal hearts (86.4 +/- 13.7) and 10 hearts with atrioventricular septal defects (22.2 +/- 26.0; p < 0.01). The outflow tract can be effectively shortened, widened, and the angle increased toward normal by augmenting the left side of the superior bridging leaflet and performing a fibromyectomy.
Standard fibromyectomy for subaortic stenosis in children with atrioventricular septal defects leads to a high rate of reoperation. Leaflet augmentation and fibromyectomy may decrease the likelihood of reoperation.
从1982年7月至1994年9月，19例患有室间隔缺损合并主动脉瓣下狭窄的患儿接受了手术治疗。具体诊断为：原发孔缺损7例，Rastelli A型缺损6例，过渡型缺损4例，流入道室间隔缺损合并腱索附着异常1例，法洛四联症合并Rastelli C型缺损1例。共进行了27次主动脉瓣下狭窄手术。3例患儿在初次室间隔缺损修复时同期进行了流出道病变的手术治疗，其余16例在修复后1.2至13.1年（平均4.9年，中位数3.9年）进行了手术。19例患儿中有18例行纤维组织切除术和心肌切除术以解除梗阻。7例患儿同期进行了左房室瓣手术。1例患儿接受了心尖主动脉管道植入术。7例患儿（36.8%）因既往治疗的主动脉瓣下狭窄需要再次手术8次。第二次手术时间为2.8至7.4年（平均4.9年）。随访时间为0.4至14.0年（中位数5.6年）。再次手术的6年精算无事件生存率为66%±15%。在10例正常心脏（86.4±13.7）和10例室间隔缺损心脏（22.2±26.0；p<0.01）中测量了流出道间隔平面与间隔嵴平面之间的夹角。通过扩大上桥瓣叶左侧并进行纤维心肌切除术，可以有效缩短、加宽流出道，并使夹角向正常方向增加。
对于室间隔缺损合并主动脉瓣下狭窄的患儿，标准的纤维心肌切除术导致再次手术率较高。瓣叶扩大术和纤维心肌切除术可能会降低再次手术的可能性。
