Department of Paediatrics, University of Melbourne, Melbourne, Australia; Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia.
Department of Paediatrics, University of Melbourne, Melbourne, Australia; Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia; Heart Research, Clinical Sciences, Murdoch Children's Research Institute, Melbourne, Australia; Department of Medicine, The Royal Melbourne Hospital, University of Melbourne, Melbourne, Australia.
Ann Thorac Surg. 2022 Jul;114(1):25-33. doi: 10.1016/j.athoracsur.2021.02.011. Epub 2021 Feb 17.
The Fontan procedure, the last of a series of palliative operations for patients born with single ventricles, is associated with a significant late burden of complications. There are other strategies for patients who are suboptimal candidates for Fontan completion; however, the long-term outcomes of these different surgical options have not been clearly elucidated. We performed a systematic literature review to establish the current role of other treatment approaches besides the Fontan procedure.
The MEDLINE and Embase databases were systematically searched for articles describing the long-term outcomes of patients with single ventricles who have not received the Fontan procedure.
A total of 36 articles met all inclusion criteria. There is a scarcity of contemporary data on the non-Fontan cohort. Historical studies provided a significant contribution.
Long-term survival of unoperated patients with single ventricles is possible under the rare conditions of having balanced hemodynamics. As many as half of patients may survive on only a systemic-to-pulmonary artery shunt or bidirectional cavopulmonary shunt for more than 20 years with reasonable functional status. In patients with a failing single ventricle, the bidirectional cavopulmonary shunt is an excellent bridge to heart transplantation and may provide better posttransplant survival than patients with a Fontan circulation. Currently, the Fontan procedure continues to be the best definitive palliation for patients born with single ventricle lesions. However, for those with borderline indications, other strategies should be carefully considered.
Fontan 手术是一系列针对单心室出生患者的姑息性手术中的最后一步，与重大的晚期并发症负担相关。对于 Fontan 完成术的非理想候选者，还有其他治疗策略；然而，这些不同手术选择的长期结果尚未明确阐述。我们进行了系统的文献综述，以确定除了 Fontan 手术之外的其他治疗方法的当前作用。
系统地在 MEDLINE 和 Embase 数据库中搜索描述未接受 Fontan 手术的单心室患者的长期结局的文章。
共有 36 篇文章符合所有纳入标准。非 Fontan 组的当代数据稀缺。历史研究提供了重要贡献。
在血流动力学平衡的罕见情况下，未手术的单心室患者长期存活是可能的。多达一半的患者可能仅通过体肺动脉分流或双向腔静脉肺动脉分流存活 20 年以上，且功能状态良好。对于患有衰竭的单心室的患者，双向腔静脉肺动脉分流术是心脏移植的极佳桥梁，并且可能比 Fontan 循环的患者提供更好的移植后生存。目前，Fontan 手术仍然是单心室病变患者的最佳根治性姑息治疗方法。然而，对于那些有边缘适应证的患者，应仔细考虑其他策略。
