title: Combined aortic and mitral valve replacement in a child with mucopolysaccharidosis type I: a case report.
abstract: Mucopolysaccaridosis syndromes are metabolic disorders that are inherited in an autosomal recessive pattern, and demonstrate cardiovascular involvement that, on rare occasions, results in surgery. The case is presented of a 12-year-old boy with symptoms and signs of congestive heart failure who was referred to the authors' department for severe mitral and aortic valvular stenosis. The patient was shown to have the somatic characteristics of mucopolysaccharidosis, while urinalysis indicated the presence of chondroitin sulfate B. Mitral and aortic valve replacements were carried out, using bileaflet mechanical valves with a standard median sternotomy and cardiopulmonary bypass. A bileaflet preservation technique was adopted during mitral valve replacement to provide better hemodynamics. Severe cardiac involvement in mucopolysaccharidosis requiring cardiac surgery in pediatrics has been rarely documented. A low-profile bileaflet mechanical valve, enabling a larger-sized valve insertion, is preferred in order to avoid patient-prosthesis mismatch during somatic growth of the patient.
authors.affiliation: Istanbul University, Istanbul Medical Faculty, Department of Cardiovascular Surgery, Istanbul, Turkey. onurgokseljet@gmail.com
authors.forename: Onur S
authors.initials: OS
authors.lastname: Goksel
authors.forename: Helin
authors.initials: H
authors.lastname: El
authors.forename: Emin
authors.initials: E
authors.lastname: Tireli
authors.forename: Enver
authors.initials: E
authors.lastname: Dayioglu
mesh_terms: D001024:Aortic Valve Stenosis / Q000209:etiology / Q000601:surgery*; D002315:Cardiopulmonary Bypass; D002648:Child; D006350:Heart Valve Prosthesis; D006801:Humans; D008297:Male; D008946:Mitral Valve Stenosis / Q000209:etiology / Q000601:surgery*; D008059:Mucopolysaccharidosis I / Q000150:complications*; D012720:Severity of Illness Index