To summarize the prevalence of the cardiovascular comorbidities in patients with amyotrophic lateral sclerosis (ALS) and explore the impacts of cardiovascular diseases on ALS.PubMed, EMBASE, OVID and Web of Science were searched systematically until July 2021 for studies on the prevalence of cardiovascular diseases among ALS patients or quantitatively investigating the effects of cardiovascular comorbidities on incidence, progression or survival of ALS. We conducted a fixed-effects or random-effects meta-analysis to calculate the summary rate or ORs (odds ratios) with 95 %CIs (confidence intervals).The comorbidity of hypertension in France (56.9%) was the highest, followed by Portugal (48%). Only 15% of Chinese ALS patients suffered from hypertension. A quarter of ALS patients in America had coronary heart disease while only 4-5% of patients with ALS in Australia or the Netherlands suffered from coronary heart disease. There was significant relationship between hypertension and survival of ALS (OR: 1.04, 95%CI: 1.01, 1.07). Coronary heart disease was considerably related to ALS onset (OR: 1.19, 95%CI: 1.14, 1.24) and heart failure could noticeably accelerate the progression rate of ALS (OR: 6.33, 95%CI: 1.55, 24.84).Cardiovascular comorbidities in ALS patients varied significantly with different regions. Hypertension could reduce the survival of ALS so the intensive treatment of chronic hypertension should be recommended to ALS patients in clinical practice. Coronary heart disease could increase the risk of ALS and heart failure was a negative prognostic factor for ALS, which deserved more attention of clinicians.Copyright © 2021 Elsevier Ltd. All rights reserved.