Transcatheter aortic valve implantation for severe aortic stenosis in a patient with mucopolysaccharidosis type II (Hunter syndrome) accompanied by severe airway obstruction.

Mucopolysaccharidosis type II, known as Hunter syndrome, is a rare inherited metabolic disorder with glycosaminoglycan accumulation leading to progressive multisystem involvement, such as heart, respiratory, and central nervous systems. In particular, concurrence of major heart and respiratory problems in this syndrome often causes difficulty in performing curative and invasive treatments. Transcatheter aortic valve implantation (TAVI) has been an established therapy for severe aortic stenosis (AS). In patients who cannot undergo surgical aortic valve replacement because of high risk for general anesthesia, TAVI with local anesthesia has become an alternative therapy for severe AS. We report herein a case of 50-year-old man with Hunter syndrome accompanied by severe airway obstruction who underwent TAVI with local anesthesia for severe AS. <Learning objective: Mucopolysaccharidosis is characterized by glycosaminoglycan accumulation leading to progressive multisystem involvement. Heart disease and respiratory problems are often concomitant in patients with mucopolysaccharidosis. When surgical treatment is required, consideration about treatment strategy and perioperative management are important because of its high surgical risk or inoperable status. We describe a case with mucopolysaccharidosis accompanied by severe airway obstruction who underwent transcatheter aortic valve implantation with local anesthesia for severe aortic stenosis.>.