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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

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.