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

Zach M DeBoard; Aaron W Eckhauser; Eric Griffiths

doi:10.1016/j.athoracsur.2017.08.027

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

Ann Thorac Surg. 2018 Jan;105(1):e31-e32. doi: 10.1016/j.athoracsur.2017.08.027.

Authors

Zach M DeBoard¹, Aaron W Eckhauser², Eric Griffiths²

Affiliations

¹ Division of Cardiothoracic Surgery, University of Utah, Salt Lake City, Utah. Electronic address: [email protected].
² Pediatric Cardiothoracic Surgery, Primary Children's Medical Center, Salt Lake City, Utah.

PMID: 29233360
DOI: 10.1016/j.athoracsur.2017.08.027

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.

Publication types

Case Reports

MeSH terms

Child, Preschool
Ehlers-Danlos Syndrome / complications
Ehlers-Danlos Syndrome / diagnostic imaging
Ehlers-Danlos Syndrome / surgery*
Humans
Male
Palliative Care*