Ozawa Yui, Asakai Hiroko, Shiraga Kazuhiro, Shindo Takahiro, Hirata Yoichiro, Hirata Yasutaka, Inuzuka Ryo
Department of Pediatrics, Chigasaki Municipal Hospital, Chigasaki, Japan.
Department of Pediatrics, University of Tokyo Hospital, Tokyo, Japan.
Pediatr Cardiol. 2019 Jun;40(5):909-913. doi: 10.1007/s00246-019-02087-2. Epub 2019 Mar 14.
Heterotaxy syndrome is associated with complex cardiac malformations and cardiac conduction system abnormalities. Those with right atrial isomerism (RAI) have dual sinus nodes and dual atrioventricular nodes predisposing them to supraventricular tachycardia (SVT). Those with left atrial isomerism (LAI) lack a normal sinus node and are at risk of sinus node dysfunction (SND) and atrioventricular block (AV block). We report the occurrence and risk factors associated with arrhythmias in heterotaxy syndrome.
A retrospective review of all heterotaxy syndrome patients born and treated at our institution between 2000 and 2014 was performed.
A total of 40 patients were identified; 16/40 (40%) with LAI and 24/40 (60%) with RAI. There were 12 deaths during follow-up [LAI 3/16 (19%), RAI 9/24 (38%); p = 0.30]. Twenty-one patients had arrhythmias during a mean follow-up period of 5.4 years; 14/16 (87%) in LAI and 7/24 (29%) in RAI (p < 0.001). Freedom from arrhythmia at 1,3,5 years of age was 75.0%, 37.9%, 22.7% in LAI, and 83.3%, 77.5%, 69.6% in RAI, respectively(p = 0.00261). LAI had a three-fold increase in developing arrhythmias. Left atrial isomerism was the only factor identified to be associated with arrhythmia occurrence.
Arrhythmias were commonly seen in heterotaxy syndrome particularly in left isomerism with more than half of the patients having arrhythmias by 3 years of age. Atrial situs was the only risk factor identified to be associated with arrhythmias, and close follow-up is warranted in these patients.
内脏反位综合征与复杂的心脏畸形及心脏传导系统异常相关。右心房异构（RAI）患者有双窦房结和双房室结，易发生室上性心动过速（SVT）。左心房异构（LAI）患者缺乏正常的窦房结，有发生窦房结功能障碍（SND）和房室传导阻滞（AV阻滞）的风险。我们报告了内脏反位综合征中心律失常的发生情况及相关危险因素。
对2000年至2014年在我们机构出生并接受治疗的所有内脏反位综合征患者进行回顾性研究。
共确定40例患者；16/40（40%）为LAI，24/40（60%）为RAI。随访期间有12例死亡[LAI 3/16（19%），RAI 9/24（38%）；p = 0.30]。在平均5.4年的随访期内，21例患者发生心律失常；LAI组14/16（87%），RAI组7/24（29%）（p < 0.001）。LAI组1岁、3岁、5岁时无心律失常的比例分别为75.0%、37.9%、22.7%，RAI组分别为83.3%、77.5%、69.6%（p = 0.00261）。LAI组发生心律失常的几率增加了两倍。左心房异构是唯一确定与心律失常发生相关的因素。
心律失常在内脏反位综合征中常见，尤其是在左心房异构患者中，超过半数患者在3岁时发生心律失常。心房位置是唯一确定与心律失常相关的危险因素，对这些患者有必要进行密切随访。
