Outcomes of left atrial isomerism over a a 28-year period at a single institution
Outcomes of left atrial isomerism over a a 28-year period at a single institution
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DOI：
10.1016/s0735-1097(00)00812-3
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发表时间：
2000-09-01
影响因子：
24
通讯作者：
Freedom, RM
中科院分区：
文献类型：
--
作者：
Gilljam, T;McCrindle, BW;Freedom, RM
我们确定了一个大的左心房异构（LAI）队列的长期结果。背景左心房异构与复杂的心脏和非心脏异常有关，这些异常可能影响预后。Kaplan-Meier生存率进行了估计，并与时间相关的死亡的独立因素进行了identified.Results有163例（63%的妇女），并注意到在36%的心外畸形，包括胆道闭锁在10%。心脏畸形包括下腔静脉中断92%，肺静脉畸形56%，房室间隔缺损49%，肺动脉闭锁或狭窄28%，主动脉缩窄16%，先天性房室传导阻滞7%。在22例心脏正常的患者中，18%死于心外畸形。在71例心脏适合双心室修复的患者中，62例（87%）接受了手术，1年生存率为80%，6年生存率为7146，10年生存率为66%，15年生存率为63%。在70例适合单心室姑息治疗的不平衡心脏缺陷患者中，47例（67%）接受了手术，1年生存率为73%，5年为61%，10年为53%，15年为48%（p < 0.001）。与时间相关的死亡的独立因素包括先天性房室传导阻滞，主动脉缩窄，单心室，胆道闭锁和其他胃肠道畸形。结论心脏和非心脏异常有助于高死亡率与LAI。心脏移植可能需要被认为是选定的高危患者的主要选择。(C)2000年，美国心脏病学会。
OBJECTIVES We determined long-term outcomes in a large cohort With left atrial isomerism (LAI).BACKGROUND Left atrial isomerism is associated with a complex spectrum of cardiac and noncardiac anomalies that may impact on outcomes.METHODS The records of all patients with LAI, born between 1970 and 1998, and treated at one center were reviewed. Kaplan-Meier survival was estimated, and independent factors associated with time-related death were identified.RESULTS There were 163 patients (63% women), and extracardiac anomalies were noted in 36%, including biliary atresia in 10%. Cardiac defects included interrupted inferior caval vein in 92%, anomalous pulmonary veins in 56%, atrioventricular septal defect in 49%, pulmonary atresia or stenosis in 28% and aortic coarctation in 16%, with congenital atrioventricular block in 7%. Of 22 patients with a normal heart, 18% died of extracardiac anomalies. Of 71 patients with hearts suitable for biventricular repair, 62 (87%) had surgery, with survival of 80% at one year, 7146 at fit.e years, 66% at 10 years and 63% after 15 years. Of 70 patients with unbalanced cardiac defects suitable for single-ventricle palliation, il 47 (67%) had surgery, with survival of 73% at one year, 61% at five years, 53% at 10 years and 48% at 15 years (p < 0.001). Independent factors associated with time-related death included congenital atrioventricular block, aortic coarctation, single ventricle, biliary atresia and other gastrointestinal malformations.CONCLUSIONS Both cardiac and noncardiac anomalies contribute to a high mortality with LAI. Cardiac transplantation may need to be a considered a primary option for selected high-risk patients. (C) 2000 by the American College of Cardiology.