A Case Report of Double Valve Replacement for Mucopolysaccharosis with Chest Pain Attack and Severe Heart Failure / 日本心臓血管外科学会雑誌

ABSTRACT

We report a successfully treated case of double valve replacement for mucopolysaccharosis in a 27-year-old woman. Mucopolysaccharosis had been suspected since she was aged 11. Symptoms of heart failure and chest pain suggested valvular disease and she was referred to us. Echocardiography, aortography and cardioangiography showed aortic regurgitation (grade IV/IV) and mitral regurgitation (grade III/IV). She received double valve replacement and was discharged on the 38th postoperative day with symptom improvement. Although urinalysis was positive for heparan-sulfate, this case could not be diagnosed definitively as mucopolysaccharosis due to normal lymphocytic enzyme-activity. However, large amounts of mucopolysaccharoid deposits were present in her removed aortic and mitral valve leaflets, and her clinical picture corresponded with mucopolysaccharosis. Thus, it was considered that her ultimate diagnosis was combined cardiac valvular disease due to mucopolysaccharosis.