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

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source: The journal of Tehran Heart Center

year: 2021

authors: Sadeghian H,Sadeghian A,Eslami B,Abbasi SH,Lotfi-Tokaldany M


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.

organization: Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran.

DOI: 10.18502/jthc.v16i1.6598

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