Intrahepatic and periduodenal embolization: New technique for Protein Losing Enteropathy treatment in congenital heart disease patients
Presented By:
Ari J Gartenberg, MD; Ganesh Krishnamurthy, MD: Abhay Srinivasan, MD; Fernando A. Escobar, MD; Jefferson N Brownell, MD; Petar Mamula, MD; Aaron G Dewitt, MD; David M Biko, MD; Erin M Pinto, CRNP; Matthew J Gillespie, MD; Michael L O’Byrne, MD, MSCE; Jonathan J Rome, MD; Yoav Dori, MD, PhD; Christopher L Smith, MD, PhD
Overview:
Background: Protein-losing enteropathy (PLE) treatment is primarily symptomatic management to reduce fluid overload and electrolyte disturbances. Liver lymphatics are a mediator of PLE pathogenesis and their embolization has shown varying success with some patients having minimal or no improvement.
Objectives: This study sought to describe extrahepatic causes of PLE using MR lymphangiography and evaluate the outcomes of a combined intrahepatic and periduodenal lymphatic embolization strategy for PLE treatment.
Methods: This was a single-center, retrospective review of imaging and interventions in 10 consecutive patients with congenital heart disease undergoing PLE treatment.
Results: Twelve lymphatic interventions were performed in 10 patients. Intrahepatic and intranodal MR lymphangiography was performed in all procedures and intramesenteric in five. Duodenal leaks were identified from intrahepatic and/or intramesenteric access in all patients. Endoscopy with injection of isosulfan blue in hepatic or periduodenal lymphatics confirmed lymphatic leak in 11/12 procedures. Glue embolization of hepatic periportal lymphatics was performed followed by targeted embolization of periduodenal lymphatics. All patients responded to a single intervention with symptom improvement and albumin ≥3g/dL (median 15d, IQR 6-20). Longer-term follow-up (median 668d, range 111-1059d) identified two populations, complete responders (n=5) and incomplete responders (n=5) with either a slow or more acute decline (<5 months) in albumin.
Conclusions: The authors demonstrate additional sources of PLE and describe a new technique targeting both the hepatic lymphatic source and periduodenal destination to treat PLE. This comprehensive strategy led to an initial improvement of albumin levels in all patients and sustained albumin levels in 50% of patients with a single intervention.
