ATS
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

Ann Thorac Surg 2009;87:625-628. doi:10.1016/j.athoracsur.2008.06.023
© 2009 The Society of Thoracic Surgeons
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Alert me to new issues of the journal
Right arrow Add to Personal Folders
Right arrow Download to citation manager
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Choi, S.
Right arrow Articles by Won, Y.
Right arrow Search for Related Content
PubMed
Right arrow Articles by Choi, S.
Right arrow Articles by Won, Y.
Related Collections
Right arrow Valve disease

Case Reports

Fabry Disease With Aortic Regurgitation

Soojeong Choi, MDa, Haesun Seo, MDa, Mooyong Park, MDa, Jinkuk Kim, MDa, Seungduk Hwang, MDa,*, Kaewon Kwon, MDb, Kyun Her, MDc, Yongsoon Won, MDc

a Department of Internal Medicine, Soonchunhyang University, Bucheon, Korea
b Department of Pathology, Soonchunhyang University, Bucheon, Korea
c Department of Thoracic and Cardiovascular Surgery, College of Medicine, Soonchunhyang University, Bucheon, Korea

Accepted for publication June 11, 2008.

* Address correspondence to Dr Hwang, Division of Nephrology, Department of Internal Medicine, Soonchunhyang University Bucheon Hospital, 1174 Jung-dong, Wonmi-Gu, Bucheon-Si, 420-767, South Korea (Email: sd7hwang{at}schbc.ac.kr).

Aortic regurgitation is not so rare in patients with Fabry disease. Enzyme replacement therapy has become the standard medical care for Fabry disease in recent years. A 31-year-old man with Fabry disease, treated with recombinant {alpha}-galactosidase enzyme replacement for 19 months was admitted for evaluation of exertional dyspnea. Cardiac workup revealed left ventricular hypertrophy, increased left ventricular size, and moderate to severe aortic regurgitation. He underwent mechanical valvular replacement and heart biopsy. Histology of his aortic valve showed myxoid degeneration of valve leaflets. His heart muscle revealed extensive hypertrophy with vacuolization and the absence of lamellar bodies. We report a case of Fabry disease with aortic regurgitation in a man who underwent valvular replacement operation during enzyme replacement therapy.






HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
ANN THORAC SURG ASIAN CARDIOVASC THORAC ANN EUR J CARDIOTHORAC SURG
J THORAC CARDIOVASC SURG ICVTS ALL CTSNet JOURNALS
Copyright © 2009 by The Society of Thoracic Surgeons.