Post-Operative Chylothorax in Patients With Congenital Heart Disease

BACKGROUND: 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. OBJECTIVES: This study aimed to report lymphatic imaging findings, determine the mechanism of chylothorax after cardiac surgery, and analyze the outcomes of lymphatic embolization. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.