Department of Cardiovascular and Thoracic Surgery, U.N. Mehta Institute of Cardiology and Research Center, B. J. Medical College, Civil Hospital Campus, Asarwa, Gujarat, India.
Department of Cardiac Anesthesiology, U.N. Mehta Institute of Cardiology and Research Center, B. J. Medical College, Civil Hospital Campus, Asarwa, Gujarat, India.
Ann Thorac Surg. 2014 Jan;97(1):196-201. doi: 10.1016/j.athoracsur.2013.07.012. Epub 2013 Sep 14.
Transposition of the great arteries with aortopulmonary window is a rare congenital cardiac anomaly. An arterial switch operation with repair of the aortopulmonary window is the preferred operation in this subset. As the tissue between the great arteries is missing, it is considered to be a complex operation. The purpose of this study is to present our experience of a simple yet highly effective surgical technique for the management of this rare complex cardiac defect.
We detail our experience of the surgery of this complex defect in 4 patients. Standard technique of an arterial switch operation with minor modification in excision of branch pulmonary arteries is all that is needed in approaching this complex subset. The moiety of tissue resulting from the absence of an aortopulmonary window was naturally covered by the proximal and distal neo-aortic flap tissue. The operative technique used in the 4 cases and their presentations are detailed in the text.
Four patients of ages 28 days, 35 days, 40 days, and 6 months were successfully operated. One patient expired on postoperative day 21. In this case, advanced age of presentation, severe pulmonary artery hypertension, and sepsis possibly caused the death. The remaining 3 patients are off medication now and are being regularly followed up.
In our experience early diagnosis and an arterial switch operation have been crucial in getting a favorable outcome in planning of this complex congenital heart disease.
大动脉转位合并主肺动脉窗是一种罕见的先天性心脏畸形。在这种情况下，动脉调转手术联合主肺动脉窗修补是首选的治疗方法。由于大动脉之间的组织缺失，因此被认为是一种复杂的手术。本研究旨在介绍我们在处理这种罕见复杂心脏缺陷方面的简单而有效的手术技术经验。
我们详细介绍了 4 例复杂畸形患者的手术经验。在接近这种复杂亚组时，只需对分支肺动脉进行微小的动脉调转手术修正，即可采用标准技术。由于缺少主肺动脉窗而产生的组织部分自然被近端和远端的新主动脉瓣组织覆盖。文中详细介绍了 4 例患者的手术技术和临床表现。
4 例患者的年龄分别为 28 天、35 天、40 天和 6 个月，均成功手术。1 例患者术后第 21 天死亡。在这种情况下，高龄、严重肺动脉高压和败血症可能导致死亡。其余 3 例患者现已停药，并定期随访。
根据我们的经验，早期诊断和动脉调转手术对于规划这种复杂先天性心脏病的良好预后至关重要。
