Quaegebeur J M, Jonas R A, Weinberg A D, Blackstone E H, Kirklin J W
Division of Cardiothoracic Surgery, College of Physician's & Surgeons of Columbia University, New York, N.Y.
J Thorac Cardiovasc Surg. 1994 Nov;108(5):841-51; discussion 852-4.
Among 326 severely symptomatic neonates with coarctation with or without ventricular septal defect, four died before an initial procedure was performed. Among the 322 undergoing an initial procedure, survival for at least 24 months was 84%; the hazard function for death was lower initially but more prolonged in patients without than in those with ventricular septal defect. Important mitral valve anomalies coexisted in 5% of patients, left ventricular hypoplasia in 5% (more commonly in patients without ventricular septal defect), narrowing of the left ventricular outflow tract in 9% (more common in patients without ventricular septal defect), and narrowing of the proximal arch in 1%; one or more of these anomalies was present in most patients without ventricular septal defect who died. Five percent of the 322 patients had more than one of these coexisting anomalies, and 8% had just one. The most commonly used technique of repair of the coarctation was resection and end-to-end anastomosis, but no technique was a risk factor for death by multivariable analysis. Extension of the area of resection so that the end-to-end anastomosis was proximal to the left subclavian artery but distal to the left common carotid artery did not increase risk; extensions beyond this, and in the case of patch graft repair, extensions proximal to the left subclavian artery, did increase risk. Patch graft repair was associated with the highest prevalence (21%) of reintervention to the coarctation repair. Among patients with coexisting moderate-sized or large ventricular septal defects, repair of the coarctation, pulmonary trunk banding, and subsequent repair of the defect were associated with the highest 2-year survival, 97% in those with single ventricular septal defect. The risk-adjusted outcomes in two institutions were less good than in all others.
在326例有或无室间隔缺损的严重症状性主动脉缩窄新生儿中，4例在首次手术前死亡。在接受首次手术的322例患儿中，至少存活24个月的比例为84%；无室间隔缺损患儿的死亡风险函数最初较低，但持续时间比有室间隔缺损的患儿更长。5%的患儿存在重要的二尖瓣异常，5%存在左心室发育不全（更常见于无室间隔缺损的患儿），9%存在左心室流出道狭窄（更常见于无室间隔缺损的患儿），1%存在近端主动脉弓狭窄；在大多数死亡的无室间隔缺损患儿中存在一种或多种这些异常。322例患儿中有5%存在不止一种这些并存异常，8%仅有一种。最常用的主动脉缩窄修复技术是切除和端端吻合，但多变量分析显示没有一种技术是死亡的危险因素。将切除范围扩大，使端端吻合位于左锁骨下动脉近端但左颈总动脉远端，不会增加风险；超出此范围，以及补片移植修复时，吻合位于左锁骨下动脉近端，则会增加风险。补片移植修复与主动脉缩窄修复再次干预的最高发生率（21%）相关。在并存中等大小或大室间隔缺损的患儿中，主动脉缩窄修复、肺动脉干环扎以及随后的缺损修复与最高的2年生存率相关，单室间隔缺损患儿的生存率为97%。两家机构经风险调整后的结果不如其他所有机构。
