Parr G V, Waldhausen J A, Bharati S, Lev M, Fripp R, Whitman V
J Thorac Cardiovasc Surg. 1983 Aug;86(2):280-7.
In a review of 126 heart specimens of simple complete transposition of the great arteries with ventricular septal defect (VSD), coarctation was noted in eight (6%). In 105 Taussig-Bing heart specimens, coarctation and/or aortic outflow tract obstruction was noted in 56 (53%) (p less than 10(-10). This was similar to our surgical experience in 26 patients with transposition and VSD or Taussig-Bing heart. Six of nine patients with Taussig-Bing heart also had coarctation of the aorta, whereas all 17 patients with transposition and VSD had a normal aorta. Four patients with Taussig-Bing heart underwent coarctation repair and pulmonary artery banding at 2 to 7 days of age. The remaining two patients with Taussig-Bing heart did not have a hemodynamically significant coarctation. Five of the six patients with Taussig-Bing heart and coarctation underwent a Senning procedure between the ages of 7 weeks and 3 1/2 years. In four (all less than 4 months of age) the VSD could not be closed through the tricuspid valve. A right ventriculotomy was done in two and contributed to their deaths. In the other two patients the VSD was left alone and the pulmonary artery banded. Both remain well. The fifth child, aged 3 1/2 years, had the VSD closed through the tricuspid valve but died in the postoperative period of renal failure. The sixth patient is awaiting further operation following coarctation repair and pulmonary artery banding. We conclude that the presence of coarctation strongly suggests that Taussig-Bing heart malformation exists rather than transposition and VSD. Pulmonary artery banding and coarctation repair are the initial procedures of choice in the management of these critically ill newborn infants. Increasing cyanosis and failure to thrive will necessitate further operation. Closure of the VSD through the tricuspid valve in these infants is difficult. Pulmonary artery banding in association with a Senning procedure is preferable to a right ventriculotomy. Closure of the VSD is then planned at a later stage.
在对126例伴有室间隔缺损（VSD）的单纯性大动脉完全转位心脏标本的回顾中，发现8例（6%）存在缩窄。在105例陶西格-宾氏心脏标本中，56例（53%）存在缩窄和/或主动脉流出道梗阻（p小于10的-10次方）。这与我们对26例大动脉转位合并VSD或陶西格-宾氏心脏患者的手术经验相似。9例陶西格-宾氏心脏患者中有6例也存在主动脉缩窄，而所有17例大动脉转位合并VSD患者的主动脉正常。4例陶西格-宾氏心脏患者在出生后2至7天接受了缩窄修复和肺动脉环扎术。其余2例陶西格-宾氏心脏患者不存在血流动力学意义上的显著缩窄。6例陶西格-宾氏心脏合并缩窄的患者中有5例在7周龄至3岁半之间接受了森宁手术。其中4例（均小于4个月龄）无法通过三尖瓣关闭室间隔缺损。2例行右心室切开术，导致患者死亡。另外2例患者未处理室间隔缺损，仅进行了肺动脉环扎术。两人情况良好。第五例患儿，3岁半，通过三尖瓣关闭了室间隔缺损，但术后死于肾衰竭。第六例患者在接受缩窄修复和肺动脉环扎术后等待进一步手术。我们得出结论，缩窄的存在强烈提示存在陶西格-宾氏心脏畸形而非大动脉转位合并VSD。肺动脉环扎术和缩窄修复是这些危重新生儿治疗的首选初始手术。青紫加重和生长发育不良将需要进一步手术。在这些婴儿中通过三尖瓣关闭室间隔缺损很困难。肺动脉环扎术联合森宁手术优于右心室切开术。随后计划在后期关闭室间隔缺损。
