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World J Pediatr Congenit Heart Surg. 2018 Oct 8:2150135118769418. doi: 10.1177/2150135118769418. [Epub ahead of print]

Arterial Switch Operation in a Patient With Ehlers-Danlos Syndrome Type IV.

Author information

1
1 Department of Cardiothoracic Surgery, Rady Children's Hospital-San Diego, San Diego, CA, USA.
2
2 UC San Diego School of Medicine, San Diego, CA, USA.

Abstract

Ehlers-Danlos syndrome vascular type IV is characterized by translucent skin, easy bruising, and fragility of arteries. A full-term female infant presented at four weeks of age with a diagnosis of d-transposition of the great arteries with restrictive atrial septal defect. She successfully underwent emergent balloon atrial septostomy and placement of patent ductus arteriosus (PDA) stent. She required restenting of the PDA and pulmonary artery banding prior to arterial switch procedure. At 16 months of age, the patient successfully underwent arterial switch procedure without complication. This report demonstrates the feasibility of an arterial switch operation along with long-term follow-up of this rare condition.

KEYWORDS:

arterial switch operation; great vessel anomaly; pathology: vascular type IV Ehlers-Danlos; vascular disease

PMID:
30296907
DOI:
10.1177/2150135118769418

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