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(Circulation. 1973;47:575.)
© 1973 American Heart Association, Inc.

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Congenital Polyvalvular Disease

SAROJA BHARATI M.D.¹ MAURICE LEV M.D.¹

¹ From the Congenital Heart Disease Research and Training Center, Hektoen Institute for Medical Research; the Departments of Pathology, Northwestern University Medical School; Pritzker School of Medicine, University of Chicago; Abraham Lincoln School of Medicine, University of Illinois; the Chicago Medical School, University of Health Sciences; and Loyola University, Stritch School of Medicine, Chicago, Illinois.

Thirty-six hearts were studied in which all valves were involved in a dysplastic process. This consisted of an increase in spongiosa with vacuolar and lacunar degeneration, and a distinct lack of elastic tissue in the proximalis and in the spongiosa. This process was similar to, but more marked than that seen in a case of a single dysplastic valve as in bicuspid aortic valve. It was completely different than that seen in hemodynamic change. These cases clinically were often associated with trisomy 18 or trisomy 13-15, and called congenital polyvalvular disease. This disease may bear some relationship to Marfan's disease and the "floppy valve".

Key Words: Dysplastic valve • Polyvalvular disease • Floppy valve • Acid mucopolysaccharides

Submitted on August 10, 1972
Accepted on October 26, 1972