Children with congenital heart disease and heterotaxy frequently have associated intestinal rotation abnormalities. The question of whether a Ladd’s procedure is indicated for asymptomatic patients remains debated. To address this, a review of the current literature was completed in January of 2023. A total of 28 relevant articles were found and reviewed. Much of the current literature is from retrospective reviews and database research resulting in low quality of evidence. The available data suggest that while the association of these anomalies is frequent, the risk of acute volvulus in these patients is low. Further, the risk of post-operative complications following prophylactic intervention, as well as the overall mortality from the patient’s underlying congenital heart disease, is fairly high in this population. Given these findings in total, an expectant management approach is pragmatic with operative intervention reserved for patients who develop gastrointestinal symptoms.