BACKGROUND: Antiphospholipid syndrome and systemic lupus erythematosus are autoimmune inflammatory conditions involving multiple organs and sharing various clinical aspects. Owing to the scarcity of data about the surgical outcomes of these autoimmune disorders, we conducted a systematic review to assess the outcomes for patients with these diagnoses undergoing heart surgery and contextualize the findings regarding high-risk cardiac surgeries. METHODS: A thorough search of PubMed, Embase and Scopus used Preferred Reporting Items for Systematic Reviews and Meta-Analyses standards to find articles that involved patients who underwent heart surgery and had antiphospholipid syndrome and systemic lupus erythematosus. Inclusion criteria concentrated on a definitive diagnosis, while case reports and studies lacking data on surgical outcomes were excluded. Using the Joanna Briggs Institute's methodologies, quality evaluation categorized studies according to their risk of bias. RESULTS: Fourteen studies with 277 patients and a prevalence of middle-aged females met the inclusion criteria out of 6381 papers. The major preoperative comorbidity in the cohort was a history of thromboembolic events (43%). Thromboembolic complications (6%) and catastrophic antiphospholipid syndrome (2%), even with appropriate anticoagulation, were notable early post-operative outcomes. Six percent of people died within 30 days. Data from follow-up studies showed a 14% death rate and a 23% frequency of thromboembolic events. CONCLUSIONS: With the striking exception of a high frequency of thromboembolic complications and catastrophic antiphospholipid syndrome, surgical results in patients with antiphospholipid syndrome and systemic lupus erythematosus are analogous to those in high-risk cardiac procedures. Improving surgical care for this susceptible population requires an understanding of these hazards.