Center for Lymphatic Imaging and Interventions and the Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Division of Cardiology, Department of Pediatrics, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.
Catheter Cardiovasc Interv. 2020 Feb;95(2):E56-E61. doi: 10.1002/ccd.28446. Epub 2019 Aug 24.
In patients with total cavopulmonary connections, elevated central venous pressures (CVP) have detrimental effects on the lymphatic system causing an imbalance in fluid production and drainage of the interstitium. This combination may result in life-threatening lymphatic complications including plastic bronchitis (PB), protein losing enteropathy (PLE), chylothorax, and ascites. While embolization of the abnormal lymphatics has greatly improved outcomes from these complications, alternative treatment strategies have been proposed that would result in improved lymphatic drainage while leaving the lymphatic system intact. We report two novel transcatheter approaches for thoracic duct (TD) decompression in two patients who developed PLE after completion of the Fontan procedure as part of staged palliation for congenital heart disease. In addition, one patient had severe concurrent PB. In both patients, a connection was created between a left superior vena cava (LSVC) to the left atrium allowing for a nonsurgical method to decompress the TD. This procedure resulted in significant clinical and laboratory improvement of both patients' PLE and other symptoms of lymphatic dysfunction.
在全腔静脉肺动脉连接患者中，升高的中心静脉压（CVP）对淋巴管系统有不利影响，导致间质液的产生和引流失衡。这种组合可能导致危及生命的淋巴并发症，包括胶质性支气管炎（PB）、蛋白丢失性肠病（PLE）、乳糜胸和腹水。虽然异常淋巴管的栓塞极大地改善了这些并发症的结局，但已经提出了替代治疗策略，这些策略将在保持淋巴管系统完整的同时改善淋巴引流。我们报告了两例新的经导管胸导管（TD）减压方法，这两例患者在 Fontan 手术完成后出现 PLE，作为先天性心脏病分期姑息治疗的一部分。此外，一名患者同时患有严重的 PB。在这两例患者中，均在左头臂静脉（LSVC）和左心房之间建立了连接，允许采用非手术方法来减压 TD。该操作使两名患者的 PLE 和其他淋巴功能障碍症状都得到了显著的临床和实验室改善。
