Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon, USA.
Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon, USA; Amsterdam University Medical Center, Amsterdam, the Netherlands.
J Am Coll Cardiol. 2022 Sep 6;80(10):951-963. doi: 10.1016/j.jacc.2022.06.020.
For patients with d-loop transposition of the great arteries (d-TGA) with a systemic right ventricle after an atrial switch operation, there is a need to identify risks for end-stage heart failure outcomes.
The authors aimed to determine factors associated with survival in a large cohort of such individuals.
This multicenter, retrospective cohort study included adults with d-TGA and prior atrial switch surgery seen at a congenital heart center. Clinical data from initial and most recent visits were obtained. The composite primary outcome was death, transplantation, or mechanical circulatory support (MCS).
From 1,168 patients (38% female, age at first visit 29 ± 7.2 years) during a median 9.2 years of follow-up, 91 (8.8% per 10 person-years) met the outcome (66 deaths, 19 transplantations, 6 MCS). Patients experiencing sudden/arrhythmic death were younger than those dying of other causes (32.6 ± 6.4 years vs 42.4 ± 6.8 years; P < 0.001). There was a long duration between sentinel clinical events and end-stage heart failure. Age, atrial arrhythmia, pacemaker, biventricular enlargement, systolic dysfunction, and tricuspid regurgitation were all associated with the primary outcome. Independent 5-year predictors of primary outcome were prior ventricular arrhythmia, heart failure admission, complex anatomy, QRS duration >120 ms, and severe right ventricle dysfunction based on echocardiography.
For most adults with d-TGA after atrial switch, progress to end-stage heart failure or death is slow. A simplified prediction score for 5-year adverse outcome is derived to help identify those at greatest risk.
对于接受心房转换术（ASO）后的右旋袢型大动脉转位（d-TGA）合并右心系统患者，需要明确导致终末期心力衰竭的风险因素。
作者旨在确定与该人群生存相关的因素。
本多中心回顾性队列研究纳入了在先天性心脏病中心就诊的、患有 d-TGA 且既往接受过 ASO 的成年人。获取了初次和最近一次就诊的临床数据。复合主要结局为死亡、移植或机械循环支持（MCS）。
在中位随访时间 9.2 年期间，1168 例患者（38%为女性，初次就诊年龄为 29 ± 7.2 岁）中，91 例（每 10 人年发生率为 8.8%）达到了研究终点（66 例死亡、19 例移植、6 例 MCS）。发生猝死/心律失常性死亡的患者比因其他原因死亡的患者更年轻（32.6 ± 6.4 岁 vs. 42.4 ± 6.8 岁；P < 0.001）。从首发临床事件到终末期心力衰竭的时间较长。年龄、房性心律失常、起搏器、双心室增大、收缩功能障碍和三尖瓣反流均与主要结局相关。主要结局的独立 5 年预测因素为既往室性心律失常、心力衰竭入院、复杂解剖、心电图 QRS 时限>120ms 和超声心动图提示严重右心功能障碍。
对于大多数接受 ASO 后的 d-TGA 成年患者，进展为终末期心力衰竭或死亡的速度较慢。本研究构建了一个简化的 5 年不良预后预测评分，有助于识别风险最高的患者。
