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Background: Familial hypercholesterolaemia (FH) is an under recognised cause of coronary artery disease, despite the proven reductions in risk with early detection and treatment., Methods: Data from 180 consecutive patients presenting to a large regional hospital with acute coronary syndrome were collected. Potential FH was assessed using the Dutch Lipid Clinic Network Criteria (DLCNC), and if patients were on statins, pre-treatment cholesterol was estimated according to a validated algorithm., Results: Ninety per cent (90%) of patients presented with non-ST elevation myocardial infarction (NSTEMI) or ST elevation myocardial infarction (STEMI). A total of 11 patients (6%) were classified as having phenotypic FH. The phenotypic FH cohort was younger (mean age 53.1 vs 62.0, p=0.011); and more likely to have documented ischaemic heart disease (63.6% vs 20.7%, p=0.001)., Phenotypic Fh Patients: Familial hypercholesterolaemia patients had a higher rate of ezetimibe use (18.2% vs 2.4%, p=0.005), but fibrate use was not significantly different. Phenotypic FH patients also had higher levels of total cholesterol, corrected LDL and triglycerides, but no statistically significant difference in HDL levels compared with non-FH counterparts., Conclusions: The prevalence of FH is relatively high among patients presenting with acute coronary syndromes. This has now been established in a regional Australian population, with similar prevalence to large European registries. This highlights the need for improved access to specialised services in regional and rural areas to reduce adverse cardiovascular (CV) outcomes., (Copyright © 2020 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)