From the Labatt Family Heart Centre (D.Y., H.N., L.G.-W., M.S., E.J., S.-J.Y.) and Department of Diagnostic Imaging (C.Z.L., L.G.-W., M.S., S.-J.Y.), The Hospital for Sick Children, Toronto, Canada; and Department of Pediatrics, University of Toronto, Canada (L.G.-W., M.S., E.J., S.-J.Y.).
Circ Cardiovasc Imaging. 2018 Feb;11(2):e006917. doi: 10.1161/CIRCIMAGING.117.006917.
It is advocated that heterotaxy should be segregated into right or left isomerism according to atrial appendage morphology. However, atrial situs determination is often based on the pattern of associated findings rather than on atrial morphology itself, which can be difficult to define. The objective was to assess how often concordant patterns of isomerism classified by atrial appendage morphology, bronchopulmonary pattern, and splenic status are breached using cardiac magnetic resonance or computed tomography. The secondary objective was to determine the feasibility of defining atrial appendage morphology using cardiac magnetic resonance or computed tomography.
Retrospective review of 114 pediatric patients (median, 2.4 years; range, 1 day-17.9 years) with heterotaxy who underwent cardiac magnetic resonance or computed tomography was performed to evaluate atrial appendage, bronchopulmonary, and visceral organ arrangements. Atrial appendage and splenic anatomy were not definable in 17 of 114 (15%) and 4 of 114 (3.5%) patients, respectively. In the remaining 93 patients, 39% had classic right isomerism (bilateral right atrial appendages, right bronchopulmonary pattern, and asplenia) and 40% had classic left isomerism (bilateral left atrial appendages, left bronchopulmonary pattern, and polysplenia). Classic pattern of isomerism was breached in 20 of 93 (21.5%) patients: 13 (65%) displayed incongruent abdominal situs, 5 (25%) incongruent bronchial situs, 1 (5%) had discrepant appendage morphology, and 1 (5%) incongruent situs at all levels.
Atrial appendage morphology is difficult to assess and not always indicative of bronchopulmonary or abdominal situs. Discordance between bronchopulmonary branching, atrial appendage arrangement, and splenic status was identified in >20% patients with heterotaxy. Independent description of each organ system is required when arrangements are disharmonious among different organ systems.
根据心耳形态，将异构分为右异构或左异构。然而，心房定位通常基于相关发现的模式，而不是心耳形态本身，这可能难以定义。目的是评估使用心脏磁共振或计算机断层扫描检查时，根据心耳形态、支气管肺模式和脾状态分类的异构一致模式违反的频率。次要目的是确定使用心脏磁共振或计算机断层扫描定义心耳形态的可行性。
回顾性分析了 114 例接受心脏磁共振或计算机断层扫描检查的异构儿童患者（中位数年龄 2.4 岁；范围 1 天至 17.9 岁），以评估心耳、支气管肺和内脏器官的排列。17/114（15%）和 4/114（3.5%）患者的心耳和脾解剖结构不可定义。在其余 93 例患者中，39%为经典右异构（双侧右心耳、右支气管肺模式和无脾），40%为经典左异构（双侧左心耳、左支气管肺模式和多脾）。20/93（21.5%）患者经典异构模式被打破：13（65%）显示不一致的腹部位置，5（25%）显示不一致的支气管位置，1（5%）心耳形态不一致，1（5%）所有级别均不一致。
心耳形态难以评估，并不总是表明支气管肺或腹部位置。异构患者中>20%存在支气管分支、心耳排列和脾状态之间的不匹配。当不同器官系统的排列不一致时，需要对每个器官系统进行独立描述。
