Loomba Rohit S, Danduran Michael, Nielsen Kim G, Ring Astrid M, Kovach Joshua, Anderson Robert H
Children's Hospital of Wisconsin/Medical College of Wisconsin, Milwaukee, WI, USA.
Rigshospitalet, Copenhage, Denmark.
Pediatr Cardiol. 2017 Feb;38(2):410-417. doi: 10.1007/s00246-016-1531-3. Epub 2016 Nov 23.
Isomerism, also known as heterotaxy, is a clinical entity that impacts multiple organ systems both anatomically and functionally. The airways and lungs are involved in a great number of these patients, leading to increased sinopulmonary symptoms, increased need for oxygenation, and increased postoperative ventilatory support. Additionally, these patients often have congenital heart disease requiring Fontan palliation. What has not been previously described, and is the focus of this study, is the results of cardiopulmonary exercise testing in those who have undergone Fontan palliation with and without isomerism. We have now compared these finding with those from patients with primary ciliary dyskinesia, as many patients with isomerism have ciliary dyskinesia. We identified patients having the Fontan circulation with and without isomerism who had undergone cardiopulmonary exercise testing, comparing the findings from healthy individuals undergoing exercise, and a comparable number of individuals with primary ciliary dyskinesia but no congenital heart disease. We were able to include a total of 68 patients in our study, with 17 in each of the four groups. Cardiopulmonary exercise testing yielded the best results in healthy patients. All patients with the Fontan circulation demonstrated mixed pulmonary disease, although those with isomerism had greater FVC and FEV1. Exercise times did not differ, although peak consumption of oxygen was greater in those with isomerism. Those with ciliary dyskinesia had only obstructive pulmonary disease and had the lowest FEF25-75 between all groups. Those with isomerism had a lesser degree of obstructive pulmonary disease when compared to those with primary ciliary dyskinesia. Patients with the Fontan circulation with and without isomerism have relatively subtle differences in their cardiopulmonary exercise testing, with both groups demonstrating restrictive lung disease. In regard to obstructive lung disease, those with isomerism tend to be more similar to the patients with primary ciliary dyskinesia than those with the Fontan circulation but without isomerism. The results are likely limited by selection bias and highlight the need for multicentric efforts to characterize cardiopulmonary exercise testing in those patients with pulmonary isomerism.
异构化，也称为内脏反位，是一种临床病症，在解剖学和功能上影响多个器官系统。大量此类患者的气道和肺部受累，导致鼻窦肺部症状增加、氧合需求增加以及术后通气支持需求增加。此外，这些患者通常患有需要进行Fontan姑息手术的先天性心脏病。此前尚未描述且本研究重点关注的是接受Fontan姑息手术且伴有或不伴有异构化的患者的心肺运动试验结果。我们现在已将这些发现与原发性纤毛运动障碍患者的发现进行了比较，因为许多异构化患者患有纤毛运动障碍。我们确定了接受过心肺运动试验且伴有或不伴有异构化的Fontan循环患者，将其结果与进行运动的健康个体以及数量相当的患有原发性纤毛运动障碍但无先天性心脏病的个体的结果进行比较。我们的研究共纳入了68名患者，四组每组各17名。心肺运动试验在健康患者中产生的结果最佳。所有Fontan循环患者均表现出混合性肺部疾病，尽管伴有异构化的患者的用力肺活量（FVC）和第1秒用力呼气量（FEV1）更高。运动时间没有差异，尽管伴有异构化的患者的峰值耗氧量更高。患有纤毛运动障碍的患者仅患有阻塞性肺部疾病，且在所有组中其75%肺活量时的用力呼气流量（FEF25 - 75）最低。与原发性纤毛运动障碍患者相比，伴有异构化的患者的阻塞性肺部疾病程度较轻。伴有或不伴有异构化的Fontan循环患者在心肺运动试验中的差异相对细微，两组均表现出限制性肺部疾病。在阻塞性肺部疾病方面，伴有异构化的患者与原发性纤毛运动障碍患者的相似性往往高于与无异构化的Fontan循环患者的相似性。这些结果可能受到选择偏倚的限制，并突出了需要多中心研究来描述那些患有肺部异构化患者的心肺运动试验特征。
