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Combined Aortic and Mitral Valve Stenosis in Mucopolysaccharidosis Syndrome Type I-S: A Report of a Rare Case



Sadeghian H1 ; Sadeghian A1 ; Eslami B1 ; Abbasi SH1, 2 ; Lotfitokaldany M1
Authors
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Authors Affiliations
  1. 1. Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Bernard Lown Scholar in Cardiovascular Health, Harvard T.H. Chan School of Public Health, Boston, MA, United States

Source: Journal of Tehran University Heart Center Published:2021

Abstract

Mucopolysaccharidosis (MPS) syndrome is an inherited metabolic disorder. In more than half of the patients with MPS syndrome, heart valve involvement is reported; however, combined aortic and mitral valve stenosis in MPS syndrome type I-S is very rare. We describe a 39-year-old man with severe mitral and aortic valve stenosis due to MPS syndrome type I-S. Transthoracic and transesophageal echocardiography revealed severe thickening and calcification in the aortic and mitral valves with severe left ventricular hypertrophy. The coronary arteries were normal in angiography. © 2021 Tehran University of Medical Sciences.