Arterial Switch Operation in a Patient With Ehlers-Danlos Syndrome Type IV. Issue 4 (July 2020)

• Record Type: Journal Article
• Authors: Murthy, Raghav; Herrick, Nicole L.; El-Said, Howaida; Grossfeld, Paul; Moore, John; Lamberti, John J.
• Is Part Of: World journal for pediatric & congenital heart surgery. Volume 11:Issue 4(2020)
• Page Start: NP182
• Page End: NP185
• Publication Date: 2020-07
• Subjects: arterial switch operation -- great vessel anomaly -- pathology: vascular type IV Ehlers-Danlos -- vascular disease; Pediatric cardiology -- Periodicals; Congenital heart disease in children -- Periodicals; Heart -- Abnormalities -- Surgery -- Periodicals; Heart -- Surgery -- Periodicals; Heart Defects, Congenital -- surgery -- Periodicals; Cardiac Surgical Procedures -- Periodicals; Child -- Periodicals; Adult -- Periodicals; 618.9212
• DOI: 10.1177/2150135118769418
• Languages: English
• ISSNs: 2150-1351

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 ofd -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.