Surgical Management of the Neonate With Heterotaxy and Long-Term Outcomes of Heterotaxy
Surgical Management of the Neonate With Heterotaxy and Long-Term Outcomes of Heterotaxy
复制标题
异位新生儿的手术治疗和异位的长期结果
DOI：
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发表时间：
2011
影响因子：
0.9
通讯作者：
R. Jonas
中科院分区：
文献类型：
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作者：
R. Jonas
对患有异位症的新生儿面临的许多挑战进行了回顾，发现完全性肺静脉畸形连接、房室瓣异常、肺动脉闭锁和心律失常（包括心脏传导阻滞）是寻求单心室途径的儿童的特殊风险因素。不同临床试验机构的经验在适用于双心室修复的患者百分比方面存在很大差异，范围从小于20%到大于50%。双心室修复可能只需要简单的异常体静脉或肺静脉的挡板，或可能涉及复杂的心室内挡板修复双出口右心室与共同房室瓣。异位的长期并发症包括Kawashima手术后肺动静脉畸形的加速发展、Fontan手术的高死亡率和发病率（尽管最近报告了改善的结果）以及晚期心律失常的发展。如果存在旋转不良，心外问题包括肠扭转的高风险，提示需要择期拉德手术。相关睫状体运动障碍的存在似乎与术后发病风险增加相关，特别是呼吸机依赖和其他呼吸系统并发症。异位儿童面临着许多挑战，往往是照顾者和家庭低估。
A review of the many challenges facing the neonate with heterotaxy has identified total anomalous pulmonary venous connection, atrioventricular valve abnormalities, pulmonary atresia, and arrhythmias including heart block as particular risk factors for the child who will pursue a single-ventricle pathway. Experience varies widely between different centers as to the percentage of patients who are suitable for biventricular repair, ranging from less than 20% to greater than 50%. Biventricular repair may only require simple baffling of anomalous systemic or pulmonary veins or may involve complex intraventricular baffle repair of double-outlet right ventricle with common atrioventricular valve. The long-term complications of heterotaxy include accelerated development of pulmonary arteriovenous malformations after the Kawashima procedure, high mortality and morbidity for the Fontan procedure (although improving results have been reported more recently), and the development of late arrhythmias. Extracardiac problems include a high risk of volvulus if malrotation is present, suggesting the need for an elective Ladd procedure. The presence of associated ciliary dyskinesia appears to be associated with an increased risk of postoperative morbidity, particularly ventilator dependence and other respiratory complications. The child with heterotaxy faces many challenges that are often underappreciated by both caregivers and families.