Fraisse A, Colan S D, Jonas R A, Gauvreau K, Geva T
Department of Cardiology, Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.
Am Heart J. 1998 Feb;135(2 Pt 1):230-6. doi: 10.1016/s0002-8703(98)70086-9.
Echocardiography has been widely used in postoperative assessment after stage I Norwood procedure, but its accuracy in detecting aortic arch obstruction (AAO) has not been determined. This study was designed to determine the accuracy of echocardiography in the diagnosis of AAO after stage I Norwood procedure, identify echocardiographic predictors of arch obstruction, and examine the time course of its development.
The records and echocardiography reports of 139 patients who survived stage I Norwood procedure were reviewed. Reference standard for the diagnosis of AAO was catheterization, surgery, or autopsy.
AAO was diagnosed by reference standard criteria in 31 (22%) patients. Echocardiography correctly diagnosed AAO in 19 patients, missed the diagnosis in five, and wrongly predicted AAO in eight, yielding a 73% sensitivity, 92% specificity, 70% positive predictive value, and 88% accuracy. Moderate or severe right ventricular dysfunction, moderate or severe tricuspid regurgitation, and an abnormal abdominal aortic Doppler flow pattern were more common in patients with AAO. The probability of AAO developing within 6 months after stage I Norwood procedure was 21.1%, with a very small likelihood after that point. Beyond the first 30 days after surgery, the risk of death was higher in patients in whom AAO developed compared with those in whom it did not (relative risk 5.9, 95% confidence interval 2.7 to 13.2).
Echocardiography is a highly specific modality in detecting AAO after stage I Norwood procedure but its sensitivity is limited. Because of the increased risk of death associated with AAO and because most obstructions develop between 1 and 6 months postoperatively, early cardiac catheterization with possible intervention should be considered in patients with moderate or severe right ventricular dysfunction, moderate or sever tricuspid regurgitation, or an abnormal abdominal Doppler flow pattern during that period.
超声心动图已广泛应用于一期诺伍德手术（Norwood procedure）后的术后评估，但在检测主动脉弓梗阻（AAO）方面的准确性尚未确定。本研究旨在确定超声心动图诊断一期诺伍德手术后AAO的准确性，识别主动脉弓梗阻的超声心动图预测指标，并研究其发展的时间进程。
回顾了139例一期诺伍德手术后存活患者的病历和超声心动图报告。AAO诊断的参考标准为心导管检查、手术或尸检。
根据参考标准，31例（22%）患者被诊断为AAO。超声心动图正确诊断了19例AAO，漏诊5例，误诊8例，敏感性为73%，特异性为92%，阳性预测值为70%，准确性为88%。中度或重度右心室功能障碍、中度或重度三尖瓣反流以及异常的腹主动脉多普勒血流模式在AAO患者中更为常见。一期诺伍德手术后6个月内发生AAO的概率为21.1%，之后可能性极小。术后30天以后，发生AAO的患者死亡风险高于未发生AAO的患者（相对风险5.9，95%置信区间2.7至13.2）。
超声心动图在检测一期诺伍德手术后的AAO方面是一种高度特异的方法，但敏感性有限。由于与AAO相关的死亡风险增加，且大多数梗阻发生在术后1至6个月之间，对于在此期间出现中度或重度右心室功能障碍、中度或重度三尖瓣反流或异常腹主动脉多普勒血流模式的患者，应考虑早期进行心脏导管检查并可能进行干预。
