Department of Thoracic and Cardiovascular Surgery, Kyung Hee University Hospital at Gangdong, Seoul, South Korea.
Division of Pediatric Cardiology, Asan Medical Center, University of Ulsan, Seoul, South Korea.
Ann Thorac Surg. 2019 Apr;107(4):1212-1217. doi: 10.1016/j.athoracsur.2018.11.052. Epub 2018 Dec 23.
Patients with double-inlet left ventricle or tricuspid atresia with transposition of the great arteries are predisposed to systemic outflow tract obstruction necessitating systemic outflow relief operations (SORO).
Between January 2000 and February 2018, 30 patients with double-inlet left ventricle (n = 20) or tricuspid atresia (n = 10) with transposition of the great arteries underwent single-ventricle palliation. Arch obstruction was observed in 14 patients. Initial palliative procedures comprised pulmonary artery banding with (n = 4) or without (n = 14) arch repair, bilateral pulmonary artery banding with ductal stenting (n = 5), primary Norwood operation (n = 4), and palliative arterial switch operation (n = 1). Cox proportional hazards model was fitted in 15 patients with initial postnatal echocardiography to identify risk factors for decreased time to SORO.
One early and one late death occurred during the median follow-up period of 66 months (10-year survival rate, 93.3%). Various types of SORO were required in 20 of 30 patients (66.7%): Damus-Kaye-Stansel procedure (n = 12), primary Norwood-type palliation (n = 4), palliative arterial switch operation (n = 1), and bulboventricular foramen extension (n = 3). Freedom from SORO at 5 years was 34.5% in all patients (N = 30). Cox regression for the subgroup (n = 15) revealed that arch obstruction (hazard ratio, 20.6; 95% confidence interval, 2.9 to 148.2; p = 0.003) and smaller systemic outflow tract area index at end-systolic phase (hazard ratio, 1.5 at 10 mm/m decrease; 95% confidence interval, 1.0 to 2.1; p = 0.033) were identified as risk factors for decreased time to SORO.
Arch obstruction and a smaller systemic outflow tract area index at end-systolic phase at initial presentation are predictors of subsequent need for SORO in patients with double-inlet left ventricle or tricuspid atresia with transposition of the great arteries.
患有左心双出口或大动脉转位伴三尖瓣闭锁的患者易发生需要行体肺分流术（SORO）的体肺流出道梗阻。
2000 年 1 月至 2018 年 2 月，30 例左心双出口（n=20）或三尖瓣闭锁（n=10）伴大动脉转位患者行单心室姑息治疗。14 例患者存在弓部梗阻。初始姑息性手术包括肺动脉环缩术伴（n=4）或不伴（n=14）弓部修复、双侧肺动脉环缩术伴导管支架（n=5）、一期 Norwood 手术（n=4）和姑息性动脉调转手术（n=1）。15 例行初始生后超声心动图检查的患者采用 Cox 比例风险模型，以明确 SORO 时间缩短的危险因素。
30 例患者中位随访 66 个月（10 年生存率 93.3%）期间，早期和晚期各死亡 1 例。30 例患者中有 20 例（66.7%）需要各种类型的 SORO：Damus-Kaye-Stansel 手术（n=12）、一期 Norwood 姑息性手术（n=4）、姑息性动脉调转手术（n=1）和卵圆孔扩大术（n=3）。所有患者（n=30）5 年 SORO 无事件生存率为 34.5%。Cox 回归亚组（n=15）分析显示，弓部梗阻（风险比 20.6；95%置信区间，2.9 至 148.2；p=0.003）和收缩末期系统流出道面积指数较小（风险比，每减少 10mm/m 降低 1.5；95%置信区间，1.0 至 2.1；p=0.033）是 SORO 时间缩短的危险因素。
大动脉转位伴左心双出口或三尖瓣闭锁患者初始表现为弓部梗阻和收缩末期系统流出道面积指数较小是需要行 SORO 的预测因素。
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