BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is associated with various cardiovascular abnormalities, including spontaneous coronary artery dissection and atrial fibrillation. However, limited data exist to describe the association between ADPKD and acute coronary syndrome or heart failure. AIMS: This systematic review and meta-analysis evaluated the occurrence of cardiovascular events among patients with ADPKD compared to those without ADPKD. METHODS: A comprehensive literature search was conducted using Embase, PubMed and Scopus databases. Of 416 initially identified articles, 18 of them were reviewed in detail, and three cohort studies, comprising a total of 7888 patients with ADPKD, were included in the final analysis. RESULTS: In comparison with patients without ADPKD, the pooled data revealed that ADPKD patients had a significantly higher odds of any adverse cardiovascular events (odds ratio (OR) 1.36
95% confidence interval (CI): 1.24-1.28), including myocardial infarction (OR 1.86
95% CI: 1.05-3.32) and congestive heart failure (OR 1.36
95% CI: 1.22-1.51). However, there was no significant difference in mortality (OR 1.37
95% CI: 0.77-2.44). CONCLUSION: Patients with ADPKD are at a significantly increased risk for major adverse cardiovascular events, such as myocardial infarction and congestive heart failure. Despite the elevated risk of these cardiovascular complications, no significant difference in overall mortality was observed. Further studies are needed to assess ADPKD's cardiovascular risk and possible preventive strategies.