O-3
Identification and Percutaneous Embolization of Abnormal Hepatopulmonary Lymphatic Connections
Presenter
Mudit Gupta, MD, PhD, Children's Hospital of Philadelphia, Philadelphia, PA
Mudit Gupta, MD, PhD1, Catherine E Tomasulo, MD, FSCAI2, Brooke Ford, BS1, Abhay S Srinivasan, MD1, Ganesh Krishnamurthy, MD DNB1, Fernando Escobar, MD1, Yoav Dori, MD, PhD1 and Christopher L Smith, MD, PhD1, (1)Children's Hospital of Philadelphia, Philadelphia, PA, (2)Inova Heart and Vascular Institute, Chesterfield, NJ
Keywords: Congenital Heart Disease (CHD), Fontan and Imaging & Physiology
Background
Plastic bronchitis (PB) and chylothorax (CTX) are lymphatic disorders previously associated with abnormal thoracic duct-pulmonary connections. We report findings of liver-thorax lymphatic connections bypassing the central lymphatic system, and results of subsequent intervention.
Methods
Single center retrospective cohort study of all patients who underwent lymphangiography with abnormal hepatopulmonary (HP) connections from 1/2016 to 1/2023. All patients with HP connections underwent bilateral inguinal intranodal and periportal hepatic lymphatic access for imaging.
Results
330 patients were screened, 46 (14%) having HP connections. 157 of 330 patients had CTX and/or PB, 44 (28%) with abnormal HP connections. Of 46 with HP connections, 31 (67%) had CTX and 19 (41%) with PB. Median age at diagnosis was 8.4 years (IQR 3.7-12.7), 38 patients with congenital heart defects (83%), including 26 (56%) single ventricle and 20 (43%) Fontan. 13 patients had prior lymphatic procedures with residual CTX and/or PB. 30 (65%) underwent glue embolization of HP connections. All targeted channels were successfully embolized. 12 patients underwent repeat lymphatic procedure including six to embolize additional HP channels and seven for non-HP interventions. Procedural complications included glue embolization in three patients with one stroke and one each of pneumothorax and pericardial effusion requiring intervention. There was no intraprocedural mortality. Care was withdrawn on a patient with hemoperitoneum who was on ECMO prior to intervention. Two patients died following transfer back to local facility. Excluding fatalities, all but one patient had improvement in PB casts or pleural effusion following HP embolization with median follow-up of 97 days (IQR 22-339). Chest tube duration for CTX patients was median 18 days (IQR 7-38) following intervention. Excluding transfers, median ICU stay was 3 days (IQR 1.5-5 days) and hospital stay was median 11 days (IQR 8-34 days).
Conclusions
We demonstrate HP connections to be a significant cause of chylothorax and plastic bronchitis. Selective embolization is a durable treatment that can decrease effusions and cast formation. Liver lymphatic imaging is recommended for CTX/PB patients.