Cardiology in the Young

Brief Reports

Aortic stenosis in a patient with Hurler's syndrome after bone marrow transplantation

Naruhito Watanabea1, Petros V. Anagnostopoulosa1 and Anthony Azakiea1 c1

a1 Department of Surgery, Division of Pediatric Cardiac Surgery, UCSF Benioff Children's Hospital, University of California San Francisco, San Francisco, California, United States of America


We describe a case of severe aortic stenosis in a 16-year-old male with Hurler's syndrome who had prior bone marrow transplantation. The excised aortic valve leaflets showed characteristic pathologic findings of Hurler's syndrome. This is the first case report of aortic valve replacement in a patient with Hurler's syndrome treated with bone marrow transplantation that demonstrates progression of the aortic valve disease despite treatment.

(Received August 31 2010)

(Accepted December 01 2010)

(Online publication January 25 2011)