Matsuda Hikaru, Ichikawa Hajime, Ueno Takayoshi, Sawa Yoshiki
Professor Emeritus Osaka University, Cardiovascular Surgery, Higashi-Takarazuka Satoh Hospital, Nagao-cho 2-1, Takarazuka, Hyogo, Japan.
Division of Pediatric Cardiac Sergey, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
Gen Thorac Cardiovasc Surg. 2017 Jun;65(6):309-320. doi: 10.1007/s11748-017-0777-x. Epub 2017 Apr 24.
Increased survival rates after corrective or palliative surgery for complex congenital heart disease (CHD) in infancy and childhood are now being coupled with increased numbers of patients who survive to adulthood with various residual lesions or sequelae. These patients are likely to deteriorate in cardiac function or end-organ function, eventually requiring lifesaving treatment including heart transplantation. Although early and late outcomes of heart transplantation have been improving for adult survivors of CHD, outcomes and pretransplant management could still be improved. Survivors of Fontan procedures are a vulnerable cohort, particularly when single ventricle physiology fails, mostly with protein-losing enteropathy and hepatic dysfunction. Therefore, we reviewed single-institution and larger database analyses of adults who underwent heart transplantation for CHD, to enable risk stratification by identifying the indications and outcomes. As the results, despite relatively high early mortality, long-term results were encouraging after heart transplantation. However, further investigations are needed to improve the indication criteria for complex CHD, especially for failed Fontan. In addition, the current system of status criteria and donor heart allocation system in heart transplantation should be arranged as suitable for adults with complex CHD. Furthermore, there is a strong need to develop ventricular assist devices as a bridge to transplantation or destination therapy, especially where right-sided circulatory support is needed.
婴儿期和儿童期复杂先天性心脏病（CHD）矫正或姑息手术后生存率的提高，如今伴随着成年后伴有各种残余病变或后遗症的存活患者数量的增加。这些患者的心脏功能或终末器官功能可能会恶化，最终需要包括心脏移植在内的挽救生命的治疗。尽管CHD成年幸存者心脏移植的早期和晚期结果一直在改善，但结果和移植前管理仍可进一步优化。接受Fontan手术的幸存者是一个脆弱的群体，尤其是当单心室生理功能衰竭时，主要表现为蛋白丢失性肠病和肝功能障碍。因此，我们回顾了对因CHD接受心脏移植的成年人的单机构和更大规模数据库分析，以便通过确定适应症和结果进行风险分层。结果显示，尽管早期死亡率相对较高，但心脏移植后的长期结果令人鼓舞。然而，需要进一步研究以改善复杂CHD的适应症标准，尤其是针对失败的Fontan手术。此外，心脏移植中目前的状态标准系统和供体心脏分配系统应进行调整，以适合患有复杂CHD的成年人。此外，迫切需要开发心室辅助装置作为移植的桥梁或终末治疗手段，特别是在需要右侧循环支持的情况下。
