Background: Arrhythmias in adult congenital heart disease (ACHD) are responsible for the majority of hospital admissions and 20–25% of late deaths. Since need for further cardiac operations is frequent in ACHD, concomitant arrhythmia surgery represents a strategic treatment modality.

Material and Methods: A two-center retrospective study was undertaken on cryoablation of supraventricular arrhythmias in 25 conescutive ACHD patients (16/9, M/F, median age 38.5 years, IQR 38–60) operated between 01/2017 and 12/2020. Nineteen (76%) had undergone one or more previous cardiac operations and 8 (32%) one or more trans-catheter ablations. Indications included Fontan conversion in seven patients, septal defect repair in 6, pulmonary valve replacement in 10 and tricuspid surgery in 2. Open-heart cryoablation included: 4 cavotricuspid isthmus ablations, 19 right-sided Maze for atrial tachycardia/flutter, and 2 Cox-Maze III for atrial fibrillation.

Results: There were 2 (8%) hospital deaths, unrelated to cryoablation, due to low cardiac output syndrome. There were no intraoperative complications related to cryoablation. Seven (28%) patients required pace-maker implantation due to post-operative atrioventricular block. All patients were discharged on oral antiarrhythmic and anticoagulantion for 6 months. After a median follow-up of 14 months (IQR 7–27) no late mortality was observed. At follow-up, 16/23 (69%) patients are in stable sinus rhythm, 12 without anti-arrhythmic therapy. Two (8.6%) patients had relapse of arrhythmia. Freedom from arrhythmia was 90.9% and cumulative risk of recurrence was 9.6%.

Conclusions: Intraoperative cryoablation is safe and effective procedure. Surgical treatment of arrhythmias should always be considered in ACHD, whenever further open-heart repair is needed.

背景：成人先天性心脏病 (ACHD) 的心律失常是大多数入院率和 20-25% 的晚期死亡的原因。由于 ACHD 患者经常需要进行进一步的心脏手术，因此伴随的心律失常手术是一种战略性治疗方式。

材料与方法：在 2017 年 1 月 1 日至 2020 年 12 月 12 日期间手术的 25 名连续性 ACHD 患者（16/9，男/女，中位年龄 38.5 岁，IQR 38-60）中进行了一项双中心回顾性研究，对室上性心律失常的冷冻消融进行了研究。19 人 (76%) 曾接受过一次或多次心脏手术，8 人 (32%) 接受过一次或多次经导管消融术。适应症包括 7 名患者的 Fontan 转换，6 名患者的间隔缺损修复，10 名肺动脉瓣置换术和 2 名三尖瓣手术。 -用于心房颤动的迷宫 III。

结果：由于低心输出量综合征，有 2 例 (8%) 住院死亡与冷冻消融无关。没有与冷冻消融相关的术中并发症。7 名 (28%) 患者因术后房室传导阻滞需要植入起搏器。所有患者出院后口服抗心律失常药和抗凝药 6 个月。中位随访 14 个月（IQR 7-27）后，未观察到晚期死亡率。随访时，16/23 (69%) 患者处于稳定的窦性心律，12 名未接受抗心律失常治疗。两名 (8.6%) 患者出现心律失常复发。无心律失常率为 90.9%，累积复发风险为 9.6%。

结论：术中冷冻消融是安全有效的手术。当需要进一步的心脏直视修复时，应始终考虑对 ACHD 进行心律失常的外科治疗。