CADTH reimbursement reviews are comprehensive assessments of the clinical effectiveness and cost-effectiveness, as well as patient and clinician perspectives, of a drug or drug class. The assessments inform non-binding recommendations that help guide the reimbursement decisions of Canada's federal, provincial, and territorial governments, with the exception of Quebec. This review assesses Avalglucosidase alfa (Nexviazyme), 100 mg/vial, 20 mg/kg of body weight, administered every other week by IV infusion. Indication: Nexviazyme (avalglucosidase alfa) is an enzyme-replacement therapy indicated for the long-term treatment of patients with late-onset Pompe disease (LOPD) (acid alpha-glucosidase deficiency).