Complete heart block during anesthetic management in a patient with mucopolysaccharidosis type VII

IT is believed that complete heart block is unlikely to occur in patients without preexisting left bundle branch block. We describe the occurrence of complete heart block during attempted placement of a central venous catheter in a child with mucopolysaccharidosis (MPS) type VII (Sly syndrome) without preexisting left bundle branch block. Numerous reports of anesthesia in patients with MPS have described airway management and respiratory complications. However, cardiac problems during anesthesia in patients with MPS should he considered important because of the possibility of preexisting cardiomyopathy or coronary stenosis.