Reemtsen Brian L, Polimenakos Anastasios C, Fagan Brian T, Wells Winfield J, Starnes Vaughn A
Childrens Hospital Los Angeles and the Keck School of Medicine, Los Angeles, Calif, USA.
J Thorac Cardiovasc Surg. 2007 Dec;134(6):1406-10; discussion 1410-2. doi: 10.1016/j.jtcvs.2007.07.047. Epub 2007 Oct 26.
Neonates with Ebstein anomaly can have severe left ventricular dysfunction caused by right ventricular enlargement and ventricular septal displacement. Fenestrated right ventricular exclusion and Blalock-Taussig shunt (Starnes procedure) have been performed to mitigate this problem; however, the fate of the excluded ventricle and its effect on the systemic ventricle have yet to be documented.
Intermediate-term data have been retrospectively collected on the 12 survivors of neonatal right ventricular exclusion. Echocardiographic examinations were compared from the time of the original right ventricular exclusion and before second-stage Glenn palliation. Measurement of the Great Ormond Street ratio (area of right atrium plus atrialized right ventricle divided by area of trabeculated right ventricle plus left atrium and left ventricle), ratio of right ventricular to left ventricular area, degree of ventricular septal impingement into the left ventricle, and left ventricular shortening fraction have been documented.
In the 12 survivors of right ventricular exclusion, a decrease in the mean Great Ormond Street ratio was observed (before fenestrated right ventricular exclusion: 1.04 +/- 0.49 vs before Glenn palliation: 0.31 +/- 0.10, P = .01). The average pre-Glenn right ventricular/left ventricular ratio was substantially less than 1.0 (mean, 0.63 +/- 0.27), demonstrating right ventricular regression. The degree of left ventricular septal impingement decreased by an average of 38% (P = .008), normalizing left ventricular morphology. At the time of Glenn palliation, the left ventricular shortening fraction was normal in all patients (mean, 42% +/- 7%).
After neonatal right ventricular exclusion (Starnes procedure) for severe Ebstein anomaly, there is a reduction in right ventricular size, as demonstrated by echocardiographic evidence of a significant decrease in Great Ormond Street ratios. This regression correlates with ventricular septal realignment and normalization of left ventricular function.
患有埃布斯坦畸形的新生儿可能因右心室扩大和室间隔移位而出现严重的左心室功能障碍。已采用开窗右心室切除术和布-陶分流术（斯塔恩斯手术）来缓解这一问题；然而，被切除心室的转归及其对体循环心室的影响尚未见报道。
回顾性收集了12例接受新生儿右心室切除术幸存者的中期数据。比较了从最初右心室切除时到二期格林分流术之前的超声心动图检查结果。记录了大奥蒙德街比值（右心房面积加房化右心室面积除以小梁化右心室面积加左心房和左心室面积）、右心室与左心室面积比值、室间隔突入左心室的程度以及左心室缩短分数。
在12例右心室切除术后的幸存者中，观察到平均大奥蒙德街比值下降（开窗右心室切除术之前：1.04±0.49，格林分流术之前：0.31±0.10，P = 0.01）。格林分流术之前右心室/左心室的平均比值显著小于1.0（平均为0.63±0.27），表明右心室缩小。室间隔突入左心室的程度平均下降了38%（P = 0.008），左心室形态恢复正常。在格林分流术时，所有患者的左心室缩短分数均正常（平均为42%±7%）。
对于严重的埃布斯坦畸形，在新生儿右心室切除术（斯塔恩斯手术）后，右心室大小减小，大奥蒙德街比值显著下降的超声心动图证据证明了这一点。这种缩小与室间隔重新排列以及左心室功能正常化相关。
