Heterotaxy syndrome, being right atrial isomerism (RAI) or left atrial isomerism (LAI), often presents with Congenital Heart Disease (CHD). Intestinal abnormalities, including malrotation are common. We assessed the spectrum of gut abnormalities and their impact on medium-term outcome in a cohort of patients with fetal and postnatal diagnoses of heterotaxy syndrome.

We reviewed the cardiology records of heterotaxy syndrome patients from two centres, regarding the presence of CHD, time for cardiac intervention, presence of gastrointestinal abnormalities, and type/time of surgery. A questionnaire about gastrointestinal status was sent to patients <18 years old. Kaplan–Meier curves were derived for survival data and freedom from intervention.

Data were included for 182 patients (49 RAI and 133 LAI) of 247 identified. Questionnaires were sent to 77 families and 47 replied. CHD was present in all RAI and 61.7% of LAI cases. Thirty-eight patients had abdominal surgery (20.9%), similar for RAI and LAI (20.4% versus 21%, p> 0.99): Ladd procedure in 17 (44.7%), non-Ladd in 12 (31.5%), and both procedures in 9 (23.7%). Ten-year freedom from Ladd procedure for all was 86% for the whole cohort (RAI = 87%; LAI = 85%, p = 0.98). Freedom from any gastrointestinal surgery at 10 years was 79% for the whole cohort (RAI = 77%; LAI = 80%, p = 0.54). Ten-year freedom from cardiac surgery was 31% for the whole cohort (RAI = 6%; LAI = 43%, p < 0.0001).

In our cohort, one in five patients required abdominal surgery, mostly in their first year of life, similar for RAI and LAI. Between 1 and 10 years of follow-up, the impact of gastrointestinal abnormalities on outcome was minimal. Medium term survival was related to CHD.

异位综合征，即右心房异构（RAI）或左心房异构（LAI），通常表现为先天性心脏病（CHD）。肠道异常，包括旋转不良很常见。我们评估了一组胎儿和产后诊断为异位综合征的患者的肠道异常谱及其对中期结果的影响。

我们回顾了来自两个中心的异位综合征患者的心脏病学记录，包括冠心病的存在、心脏干预的时间、胃肠道异常的存在以及手术的类型/时间。向<18 岁的患者发送了一份关于胃肠道状况的问卷。Kaplan-Meier 曲线来源于生存数据和免于干预。

纳入了 247 名确定的 182 名患者（49 名 RAI 和 133 名 LAI）的数据。向 77 个家庭发送了问卷，有 47 个家庭作了答复。CHD 存在于所有 RAI 和 61.7% 的 LAI 病例中。38 名患者进行了腹部手术（20.9%），RAI 和 LAI 相似（20.4% 对 21%，p> 0.99）：Ladd 手术 17 人（44.7%），非 Ladd 手术 12 人（31.5%），两者都有9 (23.7%) 的程序。整个队列的 10 年 Ladd 手术自由率为 86%（RAI = 87%；LAI = 85%，p = 0.98）。整个队列在 10 年时免于任何胃肠道手术的比例为 79%（RAI = 77%；LAI = 80%，p = 0.54）。整个队列的 10 年无心脏手术率为 31%（RAI = 6%；LAI = 43%，p < 0.0001）。

在我们的队列中，五分之一的患者需要进行腹部手术，大部分是在他们生命的第一年，RAI 和 LAI 相似。在 1 到 10 年的随访中，胃肠道异常对结果的影响很小。中期生存与冠心病有关。