Toronto General Hospital, University Health Network, Toronto, Ontario, Canada.
Toronto General Hospital, University Health Network, Toronto, Ontario, Canada.
Can J Cardiol. 2022 Jul;38(7):1100-1110. doi: 10.1016/j.cjca.2022.04.017. Epub 2022 Apr 28.
The Fontan procedure is a staged palliation for various complex congenital cardiac lesions, including tricuspid atresia, pulmonary atresia, hypoplastic left heart syndrome, and double-inlet left ventricle, all of which involve a functional single-ventricle physiology. The complexity of the patients' original anatomy combined with the anatomic and physiologic consequences of the Fontan circulation creates challenges. Teens and adults living with Fontan palliation will need perioperative support for noncardiac surgery, peripartum management for labour and delivery, interventions related to their structural heart disease, electrophysiology procedures, pacemakers, cardioversions, cardiac surgery, transplantation, and advanced mechanical support. This review focuses on the anesthetic and intensive care unit (ICU) management of these patients during their perioperative journey, with an emphasis on the continuity of preintervention planning, referral pathways, and postintervention ICU management. Requests for recipes and doses of medications are frequent; however, as in normal anesthesia and ICU practice, the method of anesthesia and dosing are dependent on the presenting medical/surgical conditions and the underlying anatomy and physiologic reserve. A patient with Fontan palliation in their early 20s attending school full-time with a cavopulmonary connection is likely to have more reserve than a patient in their late 40s with an atriopulmonary Fontan at home waiting for a heart transplant. Each case will require an anesthetic and critical care plan tailored to the situation. The critical care environment is a natural extension of the anesthetic management of a patient, with complex considerations for a patient with Fontan palliation.
法乐四联症是各种复杂先天性心脏病变的分期姑息治疗方法，包括三尖瓣闭锁、肺动脉闭锁、左心发育不全综合征和双入口左心室，所有这些病变都涉及功能性单心室生理。患者原始解剖结构的复杂性，加上法乐四联症循环的解剖和生理后果，带来了挑战。接受法乐四联症姑息治疗的青少年和成年人在需要进行非心脏手术的围手术期支持、分娩和分娩期间的围产期管理、与结构性心脏病相关的干预措施、电生理程序、起搏器、心脏复律、心脏手术、移植和高级机械支持。本综述重点介绍了这些患者在围手术期期间的麻醉和重症监护病房（ICU）管理，强调了干预前规划、转诊途径和干预后 ICU 管理的连续性。经常需要药物配方和剂量的请求；然而，与正常麻醉和 ICU 实践一样，麻醉方法和剂量取决于当前的医疗/手术情况以及潜在的解剖结构和生理储备。一个 20 多岁的接受全时学校教育并具有腔肺连接的法乐四联症患者可能比一个 40 多岁的在家等待心脏移植的具有心房间隔缺损的法乐四联症患者有更多的储备。每个病例都需要根据情况制定麻醉和重症监护计划。重症监护环境是患者麻醉管理的自然延伸，对法乐四联症患者有复杂的考虑因素。
