Deal Barbara J, Costello John M, Webster Gregory, Tsao Sabrina, Backer Carl L, Mavroudis Constantine
Divisions of Cardiology and Cardiovascular-Thoracic Surgery, Departments of Pediatrics and Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Divisions of Cardiology and Cardiovascular-Thoracic Surgery, Departments of Pediatrics and Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Ann Thorac Surg. 2016 Feb;101(2):717-24. doi: 10.1016/j.athoracsur.2015.09.017. Epub 2015 Nov 18.
Atrial arrhythmias and progressive circulatory failure frequently develop in patients with a Fontan circulation. Improvement of flow dynamics and revision of the arrhythmia substrate may improve outcomes in selected patients. We sought to determine intermediate-term outcomes after Fontan conversion with arrhythmia operations and identify characteristics associated with decreased transplant-free survival.
The first 140 Fontan conversions with arrhythmia operations at a single institution were analyzed for predictors of cardiac death or transplant and incidence of arrhythmia recurrence.
The median age at the Fontan conversion operation was 23.2 years (range, 2.6 to 47.3 years). Preoperative arrhythmias were present in 136 patients: right atrial tachycardia in 48 patients, left atrial tachycardia in 21, and atrial fibrillation in 67. Freedom from cardiac death or transplant was 90% at 5 years, 84% at 10 years, and 66% at 15 years. The median age at the last follow-up among survivors was 32 years (range, 15 to 61 years). By multivariable analysis, risk factors for cardiac death or heart transplantation were a right or indeterminate ventricular morphology, cardiopulmonary bypass time exceeding 240 minutes, ascites, protein-losing enteropathy, or a biatrial arrhythmia operation at the time of conversion. Freedom from recurrence of atrial tachycardia was 77% at 10 years. Among 67 patients with atrial fibrillation undergoing biatrial arrhythmia operations, none had recurrent atrial fibrillation.
Freedom from cardiac death or transplant for patients undergoing Fontan conversion with an arrhythmia operation is 84% at 10 years. The effects of atrial arrhythmia operations are durable in most patients. These outcomes may serve as useful benchmarks for alternative management strategies.
在接受Fontan循环手术的患者中，房性心律失常和进行性循环衰竭经常发生。改善血流动力学和修正心律失常基质可能会改善部分患者的预后。我们试图确定Fontan转换联合心律失常手术的中期预后，并确定与无移植生存时间缩短相关的特征。
对单一机构进行的首批140例Fontan转换联合心律失常手术进行分析，以确定心脏死亡或移植的预测因素以及心律失常复发的发生率。
Fontan转换手术时的中位年龄为23.2岁（范围为2.6至47.3岁）。136例患者术前存在心律失常：48例为右房性心动过速，21例为左房性心动过速，67例为房颤。5年时无心脏死亡或移植的生存率为90%，10年时为84%，15年时为66%。幸存者末次随访时的中位年龄为32岁（范围为15至61岁）。多变量分析显示，心脏死亡或心脏移植的危险因素包括右心室或不确定心室形态、体外循环时间超过240分钟、腹水、蛋白丢失性肠病或转换时进行双房心律失常手术。房性心动过速复发的10年无复发生存率为77%。在67例行双房心律失常手术的房颤患者中，无一例出现房颤复发。
接受Fontan转换联合心律失常手术的患者10年时无心脏死亡或移植的生存率为84%。房性心律失常手术对大多数患者的效果持久。这些结果可作为替代治疗策略的有用参考标准。
