Successful Percutaneous Lymphatic Intervention for Chylothorax after Pediatric Heart Transplantation

Abstract

Purpose Chylothorax after pediatric heart transplantation (HTX) is a highly morbid and challenging disease often refractory to therapies including thoracic duct ligation or pleurodesis. We report our novel approach to lymphatic imaging and percutaneous intervention for chylothorax after HTx in children. Methods We reviewed our center's data on children with post-HTx chylothorax who underwent dynamic contrast-enhanced magnetic resonance lymphangiography (DCRL) and lymphatic embolization between November 2014 and November 2017. Results Four children (50% male, median age 5 ± 45 months) were referred for post-HTx chylothorax. Three underwent HTx for congenital heart disease (including 1 single ventricle) and 1 for dilated cardiomyopathy. Three had chylothorax immediately post-HTx associated with superior caval anastomosis obstruction or diastolic dysfunction and 1 developed chylothorax 6 months post-HTx during antibody-mediated rejection. Lymphatic imaging demonstrated abnormal pulmonary lymphatic perfusion syndrome in all 4 patients, with percutaneous interventions performed including thoracic duct or channel embolization (Figure: right - fluoroscopic image of contrast injected into thoracic duct; left - DCMRL inguinal lymph node injection; arrow highlight abnormal pulmonary perfusion; arrowhead identify thoracic duct). All 4 patients with chylothorax had complete resolution with a median 6.5 days from intervention to chest tube removal. Conclusion Chylothorax after pediatric HTx may result from abnormal pulmonary lymphatic perfusion that can be successfully treated with percutaneous lymphatic embolization. Factors associated with pulmonary lymphatic diseases after pediatric HTx include congenital heart disease and elevated central venous pressure due to SVC obstruction or rejection-mediated diastolic dysfunction. Chylothorax after pediatric heart transplantation (HTX) is a highly morbid and challenging disease often refractory to therapies including thoracic duct ligation or pleurodesis. We report our novel approach to lymphatic imaging and percutaneous intervention for chylothorax after HTx in children. We reviewed our center's data on children with post-HTx chylothorax who underwent dynamic contrast-enhanced magnetic resonance lymphangiography (DCRL) and lymphatic embolization between November 2014 and November 2017. Four children (50% male, median age 5 ± 45 months) were referred for post-HTx chylothorax. Three underwent HTx for congenital heart disease (including 1 single ventricle) and 1 for dilated cardiomyopathy. Three had chylothorax immediately post-HTx associated with superior caval anastomosis obstruction or diastolic dysfunction and 1 developed chylothorax 6 months post-HTx during antibody-mediated rejection. Lymphatic imaging demonstrated abnormal pulmonary lymphatic perfusion syndrome in all 4 patients, with percutaneous interventions performed including thoracic duct or channel embolization (Figure: right - fluoroscopic image of contrast injected into thoracic duct; left - DCMRL inguinal lymph node injection; arrow highlight abnormal pulmonary perfusion; arrowhead identify thoracic duct). All 4 patients with chylothorax had complete resolution with a median 6.5 days from intervention to chest tube removal. Chylothorax after pediatric HTx may result from abnormal pulmonary lymphatic perfusion that can be successfully treated with percutaneous lymphatic embolization. Factors associated with pulmonary lymphatic diseases after pediatric HTx include congenital heart disease and elevated central venous pressure due to SVC obstruction or rejection-mediated diastolic dysfunction.