Redmond J M, Silove E D, De Giovanni J V, Wright J G, Sreeram N, Brawn W J, Sethia B
Heart Unit, Birmingham Children's Hospital, Ladywood, UK.
Eur J Cardiothorac Surg. 1996;10(11):991-5. doi: 10.1016/s1010-7940(96)80402-7.
Major associated cardiac anomalies are known to increase the risk of repair of complete atrioventricular septal defects (CAVSDs). The purpose of this study was to examine the effects of such anomalies on the current surgical management of CAVSDs and their influence on outcome following repair.
We performed a retrospective review of a 100 consecutive non-isomeric patients undergoing repair of CAVSD at our institution, between January 1989 and December 1994; patients with partial or intermediate defects were excluded. Complete atrioventricular septal defect patients with other major cardiac abnormalities (complex) were then compared to those with isolated CAVSDs.
There were 15 patients (15%) with associated anomalies; 3 had tetralogy of Fallot, 1 patient had pulmonary atresia, 6 had hypoplastic left or right ventricle, 1 had tetralogy of Fallot and hyperplastic right ventricle, 2 patients had double outlet right ventricle, 1 had hypoplastic aortic arch and 1 patient had aortic coarctation. The median age at operation was similar for both groups (4.2 months), while the median weight was not significantly different for isolated CAVSDs compared to complex (4.2 months vs 3.4 months, P = 0.89), but there was a higher incidence of trisomy 21 (70/85, 82% vs 8/15, 53.3%, P = 0.01). Two of the 85 isolated CAVSD patients (2.3%) had undergone palliative pulmonary artery banding, while 5 of the 15 complex patients (33.3%) had either banding or Blalock-Taussig shunts performed. The technique of CAVSD repair was identical in each group. All complex patients had standard repair of their associated anomalies. Hospital mortality was higher in the complex group (3/15, 20% vs 2/85, 2.3%, P = 0.004); all early deaths in the complex group occurred in patients with a hypoplastic ventricle. Reoperation for left atrioventricular valve regurgitation was required in six isolated CAVSD patients (7.1%) and in one complex (6.6%).
In the absence of significant ventricular hypoplasia, the early results of surgical repair in patients with CAVSDs and associated cardiovascular anomalies are similar to those achieved in patients with isolated CAVSD.
已知主要相关心脏异常会增加完全性房室间隔缺损（CAVSD）修复的风险。本研究的目的是探讨此类异常对CAVSD当前外科治疗的影响及其对修复后结局的影响。
我们对1989年1月至1994年12月在我院连续接受CAVSD修复的100例非异构患者进行了回顾性研究；排除部分或中间型缺损患者。然后将合并其他主要心脏异常（复杂型）的完全性房室间隔缺损患者与孤立性CAVSD患者进行比较。
有15例患者（15%）合并相关异常；3例患有法洛四联症，1例患有肺动脉闭锁，6例左心室或右心室发育不全，1例患有法洛四联症和右心室增生，2例患有右心室双出口，1例患有主动脉弓发育不全，1例患有主动脉缩窄。两组患者的手术中位年龄相似（4.2个月），孤立性CAVSD患者与复杂型患者的中位体重无显著差异（4.2个月对3.4个月，P = 0.89），但21三体综合征的发生率更高（70/85，82%对8/15，53.3%，P = 0.01）。85例孤立性CAVSD患者中有2例（2.3%）接受了姑息性肺动脉环扎术，而15例复杂型患者中有5例（33.3%）进行了环扎术或布-塔分流术。每组CAVSD修复技术相同。所有复杂型患者均对其相关异常进行了标准修复。复杂型组的医院死亡率更高（3/15，20%对2/85，2.3%，P = 0.004）；复杂型组所有早期死亡均发生在心室发育不全的患者中。6例孤立性CAVSD患者（7.1%）和1例复杂型患者（6.6%）因左房室瓣反流需要再次手术。
在无明显心室发育不全的情况下，合并心血管异常的CAVSD患者的外科修复早期结果与孤立性CAVSD患者相似。
