Department of Pediatric Cardiac Surgery and Congenital Heart Disease, National Institute of Cardiology Ignacio Chávez, Mexico City, México.
Department of Pediatric Cardiology, National Institute of Cardiology Ignacio Chávez, Mexico City, México.
J Card Surg. 2021 Dec;36(12):4476-4484. doi: 10.1111/jocs.15982. Epub 2021 Sep 7.
The aim of the study was to identify, determine, and analyze the clinical and surgical outcomes of patients with atrial isomerism (AI) undergoing cardiac surgery.
A retrospective study was carried out. Patients with diagnosis of AI undergoing cardiac surgery at the National Institute of Cardiology Ignacio Chávez, from January 1, 2010 and March 31, 2020 were included; demographic characteristics and perioperative conditions of the patients were considered.
Sixty-five patients were included, with an average age of 6.4 ± 4.9 years, 50.8% males. Thirty-eight (58.5%) had right atrial isomerism (RAI) and 27 (41.5%) had left atrial isomerism (LAI); univentricular physiology (78.5%) predominated. Atrioventricular septal defect (AVSD) in RAI and septal defects in LAI were identified as the main associated defects. The most common surgical procedures performed were modified Blalock-Taussig shunt (MBTS) (27.6%), MBTS with total anomalous pulmonary venous connection (TAPVC) repair (15.3%) and total cavopulmonary connection (TCPC) with an extracardiac conduit fenestrated (10.8%); 100% RAI required a univentricular approach, while in LAI it was 48.1%. Overall survival was 92.3%, with 100% survival in LAI with biventricular physiology and 86.8% in RAI with univentricular physiology.
The survival of our institution is similar to that of other referral centers, where patients with LAI had a better evolution than RAI; in addition, the univentricular approach was required in all with RAI. Patients with AI must undergo a rigorous evaluation to determine an adequate repair strategy, considering univentricular RAI with a high possibility of morbidity and mortality.
本研究旨在确定、确定和分析接受心脏手术的心房异构(AI)患者的临床和手术结果。
进行了一项回顾性研究。纳入 2010 年 1 月 1 日至 2020 年 3 月 31 日在国家心脏病学研究所伊格纳西奥·查韦斯(National Institute of Cardiology Ignacio Chávez)接受心脏手术的 AI 诊断患者;考虑了患者的人口统计学特征和围手术期情况。
共纳入 65 例患者,平均年龄为 6.4±4.9 岁,男性占 50.8%。38 例(58.5%)为右心房异构(RAI),27 例(41.5%)为左心房异构(LAI);单心室生理学(78.5%)为主。RAI 的房室间隔缺损(AVSD)和 LAI 的间隔缺损被确定为主要相关缺陷。最常进行的手术是改良的 Blalock-Taussig 分流术(MBTS)(27.6%)、MBTS 联合完全性肺静脉连接异常(TAPVC)修复术(15.3%)和全腔静脉肺动脉连接术(TCPC)联合心外管道开窗术(10.8%);100%的 RAI 需要单心室入路,而 LAI 则为 48.1%。总生存率为 92.3%,其中双心室生理学的 LAI 生存率为 100%,单心室生理学的 RAI 生存率为 86.8%。
本机构的生存率与其他转诊中心相似,其中 LAI 患者的预后优于 RAI;此外,所有 RAI 患者均需采用单心室入路。AI 患者必须进行严格评估,以确定适当的修复策略,考虑到 RAI 单心室的高发病率和死亡率。