Abstract

We report a rare case of mitral valve stenosis secondary to Hunter syndrome, mucopolysaccharoidosis (MPS) type II in a 33-year-old man. Anatomical abnormalities in patients with MPS present anesthetic and surgical challenges during cardiac surgery. Management of this particular patient was complicated by excessive oral secretions and atrial fibrillation. With a detailed preoperative assessment and planning for airway management, this patient successfully underwent mitral valve replacement and had an uncomplicated hospital course. After 6 months of follow-up, the patient was still in stable condition.

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