An isogeometric topological shape optimization method is developed using the level sets and Heaviside enrichments. In the level set method, the initial domain is kept fixed and its boundary is represented by an implicit moving boundary embedded in the level set functions, which facilitates to handle complicated topological shape changes. The Heaviside enrichment improves the isogeometric analysis by adding some enrichment functions to model the internal boundaries. The proposed topological shape optimization method has several benefits: exact geometric models can be obtained using the isogeometric approach and the limitation of tensor-product patches can be overcome using the Heaviside enrichments to represent the internal voids. Even in a single patch, discontinuous displacement fields as well as smooth stress field can be obtained. Since the level sets offer the implicit moving boundary inside the domain, it is easy to represent the topological shape variations in the isogeometric analysis using Heaviside enrichments.