Burch Michael, Taylor Amy, Mehew Jennifer, Dominguez Troy, Crossland David, Lewis Clive, Taylor Rhiannon, Clift Paul, Al-Attar Nawwar, Venkateswaran Rajamiyer, Banner Nicholas
Great Ormond Street Hospital, London, UK.
Great Ormond Street Hospital, London, UK.
Int J Cardiol. 2020 May 15;307:36-40. doi: 10.1016/j.ijcard.2019.08.059. Epub 2019 Sep 6.
There are no multi-centre data on the outcomes of transplant for adult congenital heart disease (ACHD) outside of North America. The literature has identified a number of concerns for this population such as increased wait-list and early post-operative mortality. We investigated outcomes in a national cohort to see if these problems are replicated outside of America.
Adults (aged ≥16 years) undergoing primary heart transplantation 1995-2014 were identified in the UK Registry and registration, operative and post-transplantation related clinical factors were compared to non ACHD recipients.
Of 3026 adults who underwent primary heart transplantation, 134 (4.4%) had ACHD (median age 30 years; 40.3% female). For the ACHD patients listed as urgent status, the time to transplant was not significantly different to non ACHD patients and ACHD were not more likely to die or be removed from the wait list. Despite ACHD recipients having longer hospital stays (27 vs. 22 days; p = 0.003) and worse 90-day survival (79.5% vs. 86.6%; p = 0.02), long-term post-transplantation survival was not significantly different. Creatinine clearance was significantly better in ACHD patients at follow-up. In the last 10 years of our study period, all single ventricle transplants have been restricted to experienced ACHD teams, one year survival for Fontan patients was 89.5%.
The use of urgent listing appears to have benefited the ACHD group by allowing equal access to transplantation, and recent concentration of expertise for single ventricle transplants has been associated with excellent early survival.
北美以外地区尚无关于成人先天性心脏病（ACHD）移植结局的多中心数据。文献中已确定该人群存在一些问题，如等待名单增加和术后早期死亡率上升。我们调查了一个全国队列的结局，以了解这些问题在美国以外地区是否同样存在。
在英国登记处识别出1995年至2014年接受初次心脏移植的成年人（年龄≥16岁），并将登记、手术及移植后相关临床因素与非ACHD受者进行比较。
在3026例接受初次心脏移植的成年人中，134例（4.4%）患有ACHD（中位年龄30岁；40.3%为女性）。对于列为紧急状态的ACHD患者，移植时间与非ACHD患者无显著差异，且ACHD患者死亡或从等待名单中移除的可能性并不更高。尽管ACHD受者住院时间更长（27天对22天；p = 0.003）且90天生存率更低（79.5%对86.6%；p = 0.02），但移植后的长期生存率无显著差异。随访时ACHD患者的肌酐清除率明显更好。在我们研究期的最后10年，所有单心室移植均限于经验丰富的ACHD团队，Fontan患者的1年生存率为89.5%。
紧急列入名单似乎使ACHD组受益，使其能够平等获得移植机会，并且近期单心室移植专业知识的集中与出色的早期生存率相关。
