Department of Cardiovascular Surgery, Faculty of Medicine, Atakent Hospital, Acibadem Mehmet Ali Aydinlar University, İstanbul, Turkey.
Department of Pediatric Cardiology, Faculty of Medicine, Atakent Hospital, Acibadem Mehmet Ali Aydinlar University, İstanbul, Turkey.
Cardiol Young. 2022 Jul;32(7):1129-1135. doi: 10.1017/S1047951121003899. Epub 2021 Oct 1.
Laterality anomalies are almost always associated with severe cardiac anomalies. Demographic properties, type of the procedures, associated anomalies, and early and mid-term prognosis of four types of laterality anomalies were analysed.
A total of 64 consecutive patients with laterality anomalies were enrolled between July 2014 and July 2020. We grouped the patients as situs solitus dextrocardia (SSD) (n = 12; 18.7%); situs inversus (SI) (n = 16; 25%); right atrial isomerism (RAI) (n = 29; 45.3%); and left atrial isomerism (LAI) (n = 7; 10.9%). TAPVC was only present in the RAI group (31%). Incidence of mitral or tricuspid atresia was higher in the SSD group (25%). All the patients were followed up with a mean of 19.06 ± 17.6 (0.1-72) months.
Early postoperative mortality was 17 patients, among 107 procedures (15.8%). Twelve patients were in the neonatal period. All ten patients survived after isolated ductal stenting. Fourteen of the deaths were in the RAI group (48.3%). The 3-year survival rates were 85% in LAI, 78.7% in SI, 55.8% in SSD, and 38% in RAI groups. According to the multivariable Cox regression model, mechanical ventilation, kidney injury, RAI, and complex surgery in the neonatal period were independent risk factors for early mortality.
Laterality anomalies are one of the most challenging patients who commonly had univentricular physiology. The most prevalent anomaly was RAI, and RAI had the worst outcome and survival. Ductal stent is an acceptable first intervention during the neonatal period in suitable patients. Complex procedures may carry a high risk of death in the neonatal period.
侧位异常几乎总是与严重的心脏异常相关。本研究分析了 4 种侧位异常的人口统计学特征、手术类型、相关异常以及早期和中期预后。
2014 年 7 月至 2020 年 7 月，共纳入 64 例连续的侧位异常患者。我们将患者分为右位心右旋（SSD）（n=12；18.7%）、反位（SI）（n=16；25%）、右房异构（RAI）（n=29；45.3%）和左房异构（LAI）（n=7；10.9%）。只有 RAI 组存在心上型完全性肺静脉异位引流（TAPVC）（31%）。SSD 组二尖瓣或三尖瓣闭锁的发生率较高（25%）。所有患者平均随访 19.06±17.6（0.1-72）个月。
107 例手术中有 17 例（15.8%）发生术后早期死亡。12 例在新生儿期。10 例孤立性导管支架置入术后全部存活。RAI 组 14 例死亡（48.3%）。LAI 组 3 年生存率为 85%，SI 组为 78.7%，SSD 组为 55.8%，RAI 组为 38%。多变量 Cox 回归模型显示，机械通气、肾损伤、RAI 和新生儿期复杂手术是早期死亡的独立危险因素。
侧位异常是最具挑战性的患者之一，常伴有单心室生理学。最常见的异常是 RAI，其预后和生存率最差。对于合适的患者，在新生儿期进行导管支架置入术是一种可接受的初始干预措施。复杂手术可能会增加新生儿期死亡的风险。
