Valve surgery in a mucopolysaccharidosis type I patient: early prosthetic valve endocarditis

• Published in: European journal of cardio-thoracic surgery Vol. 41; no. 2; pp. 448 - 449
• Main Authors: Rocha, Rodolfo V., Alvarez, Rene J., Bermudez, Christian A.
• Format: Journal Article
• Language: English
• Published: Oxford Oxford University Press 01.02.2012
• Subjects: Aortic Valve Stenosis - surgery; Biological and medical sciences; Carbohydrates (enzymatic deficiencies). Glycogenosis; Cardiology. Vascular system; Endocardial and cardiac valvular diseases; Endocarditis - surgery; Errors of metabolism; Female; Heart; Heart Valve Diseases - surgery; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Medical sciences; Metabolic diseases; Middle Aged; Mitral Valve Stenosis - surgery; Mucopolysaccharidosis I - surgery; Pneumology; Prosthesis-Related Infections - surgery; Reoperation; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of the heart; Valve replacement; Heart disease; Cardiac surgery; Human; Prosthesis; Diseases of the osteoarticular system; Metabolic diseases; Cardiovascular disease; Patient; Mucopolysaccharidosis; Replacement; Heart valve; Enzymopathy; Genetic disease; Type; Endocardial disease; Endocarditis; Early; Valve; Carbohydrate; Circulatory system; Cardiology; Pneumology
• ISSN: 1010-7940; 1873-734X
• DOI: 10.1016/j.ejcts.2011.06.013

Mucopolysaccharidosis (MPS) are rare genetic disorders, caused by enzymatic defects that lead to abnormal glycosaminoglycan metabolism and its accumulation. Hurler-Scheie syndrome (MPS I) is associated with a deficiency of the lysosomal enzyme -L-iduronidase. Enzymatic replacement with intravenous laronidase is a frequently utilized therapeutic option. In patients with MPS I, progressive glycosaminoglycan storage in the heart can lead to valvular abnormalities; however, few surgical heart valve interventions have been reported in MPS I patients. We present an unusual case of a double-valve replacement in an MPS I patient, complicated by early infective endocarditis requiring surgical reintervention. We also present a comprehensive literature review of valve surgery in patients with MPS I and a brief summary of the most relevant surgical considerations, including valve selection and infection prevention.