A recent American College of Cardiology expert analysis reviews surgical systemic vein (innominate vein) turndown as a promising treatment for protein-losing enteropathy (PLE) in patients with Fontan circulation. The procedure relieves lymphatic congestion by diverting thoracic duct drainage to a lower-pressure atrial chamber and may offer an alternative to heart transplantation for carefully selected patients.
The article emphasizes the essential role of multimodality imaging—including MR lymphangiography, cardiac MRI/CT, echocardiography and catheterization—in patient selection, surgical planning, and postoperative surveillance. Early institutional experiences, including work from Phoenix Children’s, suggest this evolving approach could meaningfully expand treatment options for this high-risk population when guided by a multidisciplinary Fontan team.
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