Itkin Maxim, Pizarro Christian, Radtke Wolfgang, Spurrier Ellen, Rabinowitz Deborah A
Neours/Dupont Children's Hospital, Wilmington, Delaware; Penn Medicine, Perelman School of Medicine, Hospital of University of Pennsylvania, Philadelphia, Pennsylvania.
Neours/Dupont Children's Hospital, Wilmington, Delaware.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2020;23:41-47. doi: 10.1053/j.pcsu.2020.03.001.
Lymphatic complications in patients with single ventricle include plastic bronchitis, protein-losing enteropathy, and chylous pleural effusion are a source of significant morbidity and mortality with historically limited therapeutic options. Novel lymphatic imaging techniques such as intranodal lymphangiography, dynamic contrast enhanced magnetic resonance lymphangiography and liver lymphangiography have allowed visualization of the lymphatic system and discovery of the pathophysiological mechanism of these conditions. This mechanism includes the combination of 2 factors: increased lymphatic flow in patients with elevated central venous pressure and presence of the lymphatic anatomical variant that allows the lymph to flow in close proximity to the serous (pleural space in chylothorax) or mucosal (plastic bronchitis and protein losing enteropathy) surfaces. Novel minimally invasive lymphatic interventional techniques, such as thoracic duct embolization, interstitial embolization and liver lymphatic embolization have allowed the obliteration of these abnormal lymphatic networks, resulting in resolution of the symptoms. Further refinement of the imaging techniques and interventional methods have subsequently allowed better patient selection and improved long term outcome of these procedures.
单心室患者的淋巴系统并发症包括塑性支气管炎、蛋白丢失性肠病和乳糜性胸腔积液，这些并发症是导致显著发病和死亡的原因，且历来治疗选择有限。新型淋巴成像技术，如结内淋巴管造影、动态对比增强磁共振淋巴管造影和肝脏淋巴管造影，已能够可视化淋巴系统并发现这些病症的病理生理机制。该机制包括两个因素：中心静脉压升高患者的淋巴流量增加，以及存在使淋巴靠近浆膜（乳糜胸的胸膜腔）或黏膜（塑性支气管炎和蛋白丢失性肠病）表面流动的淋巴解剖变异。新型微创淋巴介入技术，如胸导管栓塞、间质栓塞和肝脏淋巴栓塞，已能够闭塞这些异常淋巴网络，从而缓解症状。随后，成像技术和介入方法的进一步完善使得患者选择更加合理，并改善了这些治疗的长期效果。
