University of Washington, Division of Cardiology, Seattle, WA, United States.
University of Washington, Division of Cardiology, Seattle, WA, United States; Seattle Children's Hospital, Division of Cardiology, Seattle, WA, United States.
Int J Cardiol. 2017 Oct 15;245:135-140. doi: 10.1016/j.ijcard.2017.07.107. Epub 2017 Aug 2.
Heart failure is the leading cause of morbidity and mortality for adults with congenital heart disease (ACHD). Many patients are ineligible for transplantation, and those who are eligible often face long wait times with high wait-list morbidity. Durable mechanical circulatory support (MCS) may be an option for many patients. This systematic review evaluates the published literature on the use of durable MCS in teenagers and adults with congenital heart disease.
A comprehensive search of MEDLINE (PubMed), EMBASE, and the Cochrane Library was performed electronically in July 2015 and updated in March 2016, guided by the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines.
Individual case reports and several case series identified 66 patients with ACHD treated with durable MCS. More than half were INTERMACS 1 or 2 at the time of implantation. Patients with Fontan repairs were more frequently classified as INTERMACS 1 or 2 (89% compared to 59% or less among other groups). Cases published after 2010 showed a trend toward less severe INTERMACS status, and patients were less likely to have received transplants by the time of reporting (31% compared to 61% prior). Durable MCS was implanted as bridge-to-transplant in 77%. Patients with Fontan repair accounted for 14% of cases.
Reports of durable MCS utilization in patients with ACHD are becoming more frequent and devices are being implanted in more stable patients. Reports are mostly case reports or small case series so reporting bias is likely and prospective protocoled reporting is needed.
心力衰竭是导致成人先天性心脏病（ACHD）发病率和死亡率的主要原因。许多患者不符合移植条件，而符合条件的患者往往面临等待时间长、等待名单发病率高的问题。耐用型机械循环支持（MCS）可能是许多患者的选择。本系统评价评估了关于耐用型 MCS 在青少年和成人先天性心脏病患者中的应用的文献。
2015 年 7 月，根据系统评价和荟萃分析的首选报告项目指南，在 MEDLINE（PubMed）、EMBASE 和 Cochrane 图书馆进行了全面的电子搜索，并于 2016 年 3 月进行了更新。
个别病例报告和几个病例系列确定了 66 例接受耐用型 MCS 治疗的 ACHD 患者。在植入时，超过一半的患者为 INTERMACS 1 或 2。接受 Fontan 修复的患者更常被归类为 INTERMACS 1 或 2（89%比其他组的 59%或更少）。2010 年后发表的病例报告显示，INTERMACS 状态的严重程度呈下降趋势，且报告时接受移植的可能性较低（31%比之前的 61%）。耐用型 MCS 作为桥接移植植入的比例为 77%。Fontan 修复患者占病例的 14%。
关于 ACHD 患者使用耐用型 MCS 的报告越来越频繁，并且在病情更稳定的患者中植入更多设备。报告主要是病例报告或小病例系列，因此可能存在报告偏倚，需要进行前瞻性的协议报告。
