Double Valve Replacement in a Patient With Hunter Syndrome.

ABSTRACT

Hunter syndrome is a rare disorder in which affected patients have significant airway abnormalities (macroglossia, small mouth opening, and supraglottic narrowing) that complicate their management. Deposition of glycosaminoglycans in the heart leads to cardiomyopathy, and cardiac valve dysplasia that can lead to valvular stenosis or regurgitation or both, necessitating valve replacement. Management of patients with Hunter syndrome is complex and needs a multidisciplinary team approach. Mechanical valve replacement is a suitable treatment option.