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

Hakimeh Sadeghian; Afsaneh Sadeghian; Bahareh Eslami; Seyed Hesameddin Abbasi; Masoumeh Lotfi-Tokaldany

doi:10.18502/jthc.v16i1.6598

Hakimeh Sadeghian Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran.
Afsaneh Sadeghian Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran.
Bahareh Eslami Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran.
Seyed Hesameddin Abbasi Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran.
Masoumeh Lotfi-Tokaldany Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran.

DOI: https://doi.org/10.18502/jthc.v16i1.6598

Keywords: Mucopolysaccharidosis I; Mitral valve stenosis; Aortic valve stenosis; Heart valve diseases

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.