Authors: , , , , , , , , , ,


The number of patients surviving repair of complex congenital heart disease (CCHD) has increased due to improved surgical techniques, post operative management and outpatient care. Likewise, this growing patient population has demonstrated an increasing number and complexity of complications involving the lymphatic system. To evaluate the peripheral and central lymphatic system, whole-body lymphangioscintigraphy (LAS) is considered as the initial imaging evaluation of choice. To date, very few publications exist on the value of lymphatic imaging techniques in infants and small children with lymphatic complications following surgery for congenital heart disease. A retrospective review of medical records from 2008 to 2018 was performed for pediatric patients referred for lymphatic complications after CCHD surgery at an academic medical center. LAS and SPECT/CT was performed using intradermal bipedal injections of Tc 99m labeled filtered sulfur colloid, and in some patients also bilateral hand injections, followed by dynamic imaging and whole- body planar imaging typically up to 180 minutes post injection. Clinical decision making and outcomes were recorded. LAS and SPECT/CT were performed without complication in pediatric patients with prior surgery for CCHD. LAS successfully localized various lymphatic abnormalities such as lymphatic obstruction, reflux, and leaks, which were further delineated by SPECT/CT. LAS findings directed further evaluation with more definitive studies, management and prognosis. Five of the ten patients had follow up outcome data – 2 years and up to 10 years. LAS and SPECT/CT are safe and effective techniques for the initial evaluation of lymphatic abnormalities in pediatric patients with CCHD. LAS, particularly with further 3D localization by SPECT/CT, provides functional imaging of peripheral and central lymphatic flow and thus provides guidance for medical therapy, non operative interventional management, and surgical therapy for these diverse, debilitating, and often life threatening disorders.

Keywords: whole-body lymphangioscintigraphy, single photon emission computed tomography SPECT/CT, congenital heart disease, Fontan repair, lymphatic complications, chylothorax, plastic bronchitis, protein losing enteropathy

How to Cite: Kuo, P. , Barber, B. , Kylat, R. , Klewer, S. , Behan, S. , Lau-Braunhut, S. , Bernas, M. , Moedano, L. , Bedrick, A. , Mustacich, D. & Witte, M. (2020) “WHOLE-BODY LYMPHANGIOSCINTIGRAPHY AND SPECT/CT IN CHILDREN WITH LYMPHATIC COMPLICATIONS AFTER SURGERY FOR COMPLEX CONGENITAL HEART DISEASE”, Lymphology. 52(4). doi: