Abstract 16011: Lymphatic Intervention Following Superior Cavo-Pulmonary Anastomosis Potentially Allows for Successful Fontan Completion

Abstract

Introduction: Superior cavopulmonary anastomosis (SCPC) inevitably exposes the thoracic duct to higher venous pressure, potentially triggering disordered lymphatic perfusion manifesting as chylothorax (CT) and/or plastic bronchitis (PB) . Though outcomes following lymphatic interventions after Fontan completion (FC) are well described, they are not well described in patients after SCPC. Methods: A single-center retrospective study of patients who underwent lymphatic intervention for PB or CT following SCPC 1/1/2011-12/31/2021 was performed, excluding those with planned two ventricle or 1.5 ventricle repair, patients who underwent thoracic duct ligation, and those in whom interventions were performed either prior to SCPC or following FC. Results: In total, 27 patients (56% male) were studied, of which 22% had PB and 78% with CT, 7% with Noonan’s syndrome and 19% with heterotaxy syndrome. Median age at intervention was 11.3 months (IQR 7.4 - 33.5). Median time following SCPC was 3.5 months (IQR 2.1 - 24.2). In terms of interventions, 41% (n=11) underwent selective lymphatic channel embolization. Of these 36% (4/11) underwent subsequent thoracic duct embolization (TDE). The remaining 59% (n=16) patients underwent TDE. The thoracic duct was able to be accessed in 23 patients (85.2%). Twenty-four patients (19/21 with CT, 5/6 with PB) had resolution of symptoms following transcatheter intervention at our center. Following intervention, seven patients (all of whom were of the type IV lymphatic subtype) were successfully referred for FC, ten are currently awaiting FC, five are not Fontan candidates and five passed away from non-procedural comorbidities. Median duration of post-operative chest tubes following FC was 7 days (IQR 6-8) and median follow up is 39.1 months (IQR 15.1 - 45.1). Conclusions: Selective lymphatic embolization and TDE are feasible in SCPC recipients with CT and PB, with most patients experiencing symptomatic improvement. Subsequent FC has been achieved in some patients with type IV lymphatic abnormalities, suggesting that these interventions may alter the unnatural history of lymphatic dysfunction in single ventricle patients. Future research is needed to define longer term outcomes of this high-risk cohort.