Kanter Kirk R, Mahle William T, Vincent Robert N, Berg Alexandria M
Division of Cardio-Thoracic Surgery, Department of Surgery, Emory University School of Medicine, Atlanta, GA, USA.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2011;14(1):24-8. doi: 10.1053/j.pcsu.2011.01.011.
Despite increasing surgical success with staged palliation of hypoplastic left heart syndrome and its variants, some of these children eventually may require cardiac transplantation. Sixteen (7.8%) of 206 children ≤18 years old undergoing primary heart transplantation had a previous Norwood palliation. Two (12.5%) developed significant aortic problems after transplantation related to the initial homograft reconstruction of the aorta. Patient 1 developed acute graft failure requiring extracorporeal membrane oxygenator support post-transplant. During acute retransplantation 2 days later, the new donor aorta was sewn to a remnant of the initial donor aorta rather than to the heavily calcified reconstructed native aorta. Two months later, the patient required reoperation for acute airway compression from an aortic pseudoaneurysm caused by necrosis of the bridge of aortic tissue from the first transplant. Patient 2 had multiple balloon dilatations of recurrent coarctation after transplantation. Eighteen years post-transplant, during work-up for chronic fever and weight loss, computerized tomography showed a mycotic aneurysm of the reconstructed transverse aorta with contained rupture necessitating removal and replacement of the entire reconstructed aorta. Although uncommon, aortic complications in pediatric heart transplant patients with previous Norwood arch reconstruction can present with unusual manifestations requiring heightened vigilance.
尽管在对左心发育不全综合征及其变异型进行分期姑息治疗方面手术成功率不断提高，但其中一些儿童最终可能仍需要进行心脏移植。在206例接受初次心脏移植的18岁及以下儿童中，有16例（7.8%）曾接受过诺伍德姑息手术。其中2例（12.5%）在移植后出现了与最初主动脉同种异体移植重建相关的严重主动脉问题。患者1在移植后发生急性移植物功能衰竭，需要体外膜肺氧合支持。在2天后进行的急性再次移植过程中，新的供体主动脉被缝合到最初供体主动脉的残余部分，而不是严重钙化的重建自体主动脉上。两个月后，患者因首次移植后主动脉组织桥坏死导致的主动脉假性动脉瘤引起急性气道压迫而需要再次手术。患者2在移植后因复发性主动脉缩窄进行了多次球囊扩张。移植18年后，在对慢性发热和体重减轻进行检查期间，计算机断层扫描显示重建的横行主动脉有一个真菌性动脉瘤，伴有内破裂，需要切除并更换整个重建主动脉。虽然不常见，但曾接受过诺伍德主动脉弓重建的小儿心脏移植患者的主动脉并发症可能会出现不寻常的表现，需要提高警惕。
