Open AccessSevere aortic stenosis in systemic lupus erythematosus and mucopolysaccharidosis type II (hunter's syndrome)
Author(s) -
Zimmermann B.,
Lally E. V.,
Sharma S. C.,
Schoen F. J.,
Kaplan S. R.
Publication year - 1988
Publication title -
clinical cardiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.263
H-Index - 72
eISSN - 1932-8737
pISSN - 0160-9289
DOI - 10.1002/clc.4960111014
Subject(s) - medicine , mucopolysaccharidosis , hurler syndrome , valvular heart disease , systemic disease , mucopolysaccharidosis type i , stenosis , autopsy , regurgitation (circulation) , hunter syndrome , aortic valve , cardiology , pathology , disease , enzyme replacement therapy
Abstract Cardiac manifestations of the mucopolysaccharidoses often include valvular regurgitation, but stenotic lesions are quite rare. This report describes a 30‐year‐old man with mucopolysaccharidosis type II (Hunter's syndrome) and systemic lupus erythematosus who developed severe progressive aortic stenosis and died. Autopsy examination revealed evidence of various cardiac mucopolysaccharide disease including valvular leaflets thickened and distorted with fibrocalcific nodules. A brief review of previously reported valvular disease in Hunter's syndrome and other mucopolysaccharidoses is presented. This is also the first report of a patient with both systemic lupus and a mucopolysaccharidosis.