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Ann Thorac Surg 2004;78:1475-1476
© 2004 The Society of Thoracic Surgeons


Case report

HLHS With Severe Aortic Insufficiency in a Patient With 45,X/46,XY Mosaicism

Muhammad A. Mumtaz, MDa,*, Roger B. B. Mee, MB, ChBa, Athar Qureshi, MDb, Chandrakant R. Patel, MDc, Adel K. Younoszai, MDb

a Departments of Pediatric and Congenital Heart Surgery, The Cleveland Clinic Children's Hospital, Cleveland, Ohio, USA
b Pediatric Cardiology, The Cleveland Clinic Children's Hospital, Cleveland,, USA
c The Heart Center, Children's Hospital Medical Center of Akron, Akron, Ohio, USA

Accepted for publication July 10, 2003.

* Address reprint requests to Dr Mumtaz, The Cleveland Clinic Foundation, M-41, 9500 Euclid Ave, Cleveland, OH, USA 44122
mumtazm{at}ccf.org

Aortic insufficiency is not a part of the hypoplastic left heart syndrome. This report describes a rare case of congenital aortic insufficiency from a detached leaflet in a patient with hypoplastic left heart syndrome and 45,X/46XY mosaicism. The patient was subsequently treated with the modified Norwood procedure along with suture closure of aortic valve.







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