Pasquini L, Sanders S P, Parness I A, Colan S D, Van Praagh S, Mayer J E, Van Praagh R
Department of Cardiology, Children's Hospital, Boston, Massachusetts 02115.
J Am Coll Cardiol. 1993 Jun;21(7):1712-21. doi: 10.1016/0735-1097(93)90392-e.
We sought to study the range of conal morphology in transposition of the great arteries with ventricular septal defect and their embryologic and surgical implications.
Conal anatomy in transposition of the great arteries and ventricular septal defect is variable and might affect surgical repair.
Conal anatomy was explored using two-dimensional echocardiography in 119 patients with transposition of the great arteries and a large ventricular septal defect who presented between 1984 and 1991. The influence of conal anatomy on surgical technique was determined by review of the operative reports. Specimens of transposition of the great arteries with unusual conal anatomy were selected from the Cardiac Registry for comparison with the echocardiograms.
One hundred five patients (88.2%) had subaortic conus only with no subpulmonary conus (Group 1). Subarterial conus was present bilaterally in eight patients (6.7%) (Group 2). Four patients (3.4%) had only subpulmonary conus with no (or minimal) subaortic conus (Group 3). Among these four patients, the aorta was posterior to the pulmonary artery in one patient, side by side relative to the pulmonary artery in two patients and slightly anterior in the fourth patient. Subarterial conus was absent bilaterally in two patients (1.7%) (Group 4); the aorta was slightly posterior in one and side by side with the pulmonary artery in the other.
This variability of conal anatomy in transposition of the great arteries with ventricular septal defect implies four mechanisms by which transposition can occur. The conal anatomy appeared to affect surgical repair in Groups 1 and 2 insofar as it influenced ventricular outflow tract obstruction. In Groups 3 and 4, an arterial switch operation was performed in four of the six patients. The posterior location of the aorta obviated the need for the Lecompte maneuver in two of these four patients. In the remaining two cases in Groups 3 and 4, the condition was repaired by directing the left ventricular outflow across the ventricular septal defect to the aorta using a patch, with or without placement of a conduit from the right ventricle to the pulmonary artery.
我们试图研究室间隔缺损合并大动脉转位时圆锥形态的范围及其胚胎学和手术意义。
大动脉转位合并室间隔缺损时的圆锥解剖结构多变，可能会影响手术修复。
对1984年至1991年间就诊的119例大动脉转位合并大型室间隔缺损患者，使用二维超声心动图探查圆锥解剖结构。通过查阅手术报告确定圆锥解剖结构对手术技术的影响。从心脏登记处选取具有异常圆锥解剖结构的大动脉转位标本，与超声心动图进行对比。
105例患者（88.2%）仅有主动脉下圆锥，无肺动脉下圆锥（第1组）。8例患者（6.7%）双侧均有动脉下圆锥（第2组）。4例患者（3.4%）仅有肺动脉下圆锥，无（或仅有少量）主动脉下圆锥（第3组）。在这4例患者中，1例患者的主动脉位于肺动脉后方，2例患者的主动脉与肺动脉并列，第4例患者的主动脉略靠前。2例患者（1.7%）双侧均无动脉下圆锥（第4组）；1例患者的主动脉略靠后，另1例患者的主动脉与肺动脉并列。
室间隔缺损合并大动脉转位时圆锥解剖结构的这种变异性提示了大动脉转位可能发生的四种机制。圆锥解剖结构似乎在第1组和第2组中影响手术修复，因为它影响心室流出道梗阻。在第3组和第4组中，6例患者中有4例接受了动脉调转术。在这4例患者中的2例，主动脉的后方位置使得无需进行Lecompte maneuver操作。在第3组和第4组的其余2例病例中，通过使用补片将左心室流出道穿过室间隔缺损引向主动脉，无论是否放置从右心室到肺动脉的导管来修复病情。
