Children's Hospital of Philadelphia, Division of Cardiology, Philadelphia, Pennsylvania.
Children's Hospital of Philadelphia / Hospital of the University of Pennsylvania, Center for Lymphatic Imaging and Interventions, Philadelphia, Pennsylvania.
J Am Coll Cardiol. 2017 May 16;69(19):2410-2422. doi: 10.1016/j.jacc.2017.03.021.
Post-operative chylothorax in patients with congenital heart disease is a challenging problem with substantial morbidity and mortality. Currently, the etiology of chylothorax is poorly understood and treatment options are limited.
This study aimed to report lymphatic imaging findings, determine the mechanism of chylothorax after cardiac surgery, and analyze the outcomes of lymphatic embolization.
We conducted a retrospective review of 25 patients with congenital heart disease and post-operative chylothorax who presented for lymphatic imaging and intervention between July 2012 and August 2016.
Based on dynamic contrast-enhanced magnetic resonance lymphangiography and intranodal lymphangiography, we identified 3 distinct etiologies of chylothorax: 2 patients (8%) with traumatic leak from a thoracic duct (TD) branch, 14 patients (56%) with pulmonary lymphatic perfusion syndrome (PLPS), and 9 patients (36%) with central lymphatic flow disorder (CLFD), the latter defined as abnormal central lymphatic flow, effusions in more than 1 compartment, and dermal backflow. Patients with traumatic leak and PLPS were combined into 1 group of 16 patients without CLFD, of whom 14 (88%) had an intact TD. Sixteen patients underwent lymphatic intervention, including complete TD embolization. All 16 patients had resolution of chylothorax, with a median of 7.5 days from intervention to chest tube removal and 15 days from intervention to discharge. The 9 patients with CLFD were considered a separate group, of whom 3 (33%) had an intact TD. Seven patients underwent lymphatic intervention but none survived.
Most patients in this study had nontraumatic chylothorax and dynamic contrast-enhanced magnetic resonance lymphangiography was essential to determine etiology. Lymphatic embolization was successful in patients with traumatic leak and PLPS and, thus, should be considered first-line treatment. Interventions in patients with CLFD were not successful to resolve chylothorax and alternate approaches need to be developed.
先天性心脏病患者术后乳糜胸是一个具有高发病率和死亡率的棘手问题。目前，乳糜胸的病因尚不清楚，治疗选择有限。
本研究旨在报告淋巴成像结果，确定心脏手术后乳糜胸的发病机制，并分析淋巴栓塞的治疗效果。
我们回顾性分析了 2012 年 7 月至 2016 年 8 月期间因淋巴成像和介入而就诊的 25 例先天性心脏病术后乳糜胸患者。
基于动态对比增强磁共振淋巴造影和淋巴结内淋巴造影，我们确定了 3 种不同的乳糜胸病因：2 例（8%）患者为胸导管（TD）分支外伤性漏，14 例（56%）患者为肺淋巴灌注综合征（PLPS），9 例（36%）患者为中央淋巴流障碍（CLFD），后者定义为中央淋巴流异常、1 个以上部位积液和皮肤回流。外伤性漏和 PLPS 患者被归入 1 组共 16 例，无 CLFD，其中 14 例（88%）TD 完整。16 例患者接受了淋巴介入治疗，包括完全 TD 栓塞。16 例患者均治愈乳糜胸，从介入治疗到拔胸管的中位时间为 7.5 天，从介入治疗到出院的中位时间为 15 天。9 例 CLFD 患者被视为单独一组，其中 3 例（33%）TD 完整。7 例患者接受了淋巴介入治疗，但均未存活。
本研究中大多数患者为非外伤性乳糜胸，动态对比增强磁共振淋巴造影对确定病因至关重要。外伤性漏和 PLPS 患者的淋巴栓塞治疗效果良好，因此应作为一线治疗方法。CLFD 患者的介入治疗未能治愈乳糜胸，需要开发替代方法。
