Resolution of Protein-Losing Enteropathy after Congenital Heart Disease Repair by Selective Lymphatic Embolization

Abstract

With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, post-double switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ≤0.9 g/dL) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.

Pathophysiology, Diagnosis, and Management of Canine Intestinal Lymphangiectasia: A Comparative Review

“…Following treatment, his serum albumin concentration improved from 1.9 g/dL to 5.0 g/dL over a period of 8 months [ 106 ]. Embolization is also commonly performed after the Fontan procedure or in patients with right heart failure and IL [ 64 , 107 , 108 ]. For more extensive disease, small numbers of reports have described positive responses to corticosteroids, octreotide, sirolimus or everolimus, propranolol [ 109 , 110 ], and tranexamic acid [ 97 , 111 ].…”

Management of circulatory failure after Fontan surgery

“…Lymphatic interventions for patients with lymphatic disease can also be attempted. Thoracic duct embolization and selective lymphatic embolization have been used to successfully treat patients with lymphatic insufficiency ( 75 – 77 ). Our institution prefers to use selective lymphatic embolization as it leaves the thoracic duct intact for other possible interventions in the future ( 64 ).…”

Tricuspid atresia: Where are we now?

“…Postoperative protein‐losing enteropathy can be treated using the dietary limitation of long‐chain triglycerides, medications including diuretics and corticosteroids, as well as surgical management, such as a Fontan fenestration 57 . More recently, it has been shown that selective glue embolization of leaking intestinal lymphatic trunks led to a prompt reversal of PLE 66 . Third, post‐Fontan patients are at a high risk of thrombus formation.…”

“…B, Original Fontan procedure. C, Intra-atrial lateral tunnel Fontan technique.D, Extracardiac conduit Fontan technique with fenestrationMore recently, it has been shown that selective glue embolization of leaking intestinal lymphatic trunks led to a prompt reversal of PLE 66. Third, post-Fontan patients are at a high risk of thrombus formation.…”