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Single-Ventricle Palliation in a 4-Year-Old With Ehlers-Danlos Syndrome
Zach M DeBoard1, Aaron W Eckhauser2, Eric Griffiths2
1Division of Cardiothoracic Surgery, University of Utah, Salt Lake City, Utah.
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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