Single-Ventricle Palliation in a 4-Year-Old With Ehlers-Danlos Syndrome.

DeBoard, Zach M; Eckhauser, Aaron W; Griffiths, Eric · Ann Thorac Surg · 2018

case_report · Level V

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Abstract

We report the case of a 4-year-old boy with Ehlers-Danlos syndrome undergoing single-ventricle palliation for an unbalanced atrioventricular canal defect. No reports of single-ventricle palliation in the setting of connective tissue disorders exist in the current literature. Unique findings on the patient's preoperative imaging included a disproportionately large neoaortic root and a regurgitant atrioventricular valve, which may foretell the need for future intervention.

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