{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T01:06:06Z","timestamp":1791421566771,"version":"4.3.3"},"reference-count":25,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"6","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["ATVB"],"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:sec>\n                    <jats:title>BACKGROUND:<\/jats:title>\n                    <jats:p>Patients with homozygous familial hypercholesterolemia (HoFH) remain at very high cardiovascular risk despite the best standard of care lipid-lowering treatment. The addition of evinacumab, an angiopoietin-like protein 3 monoclonal antibody, more than halves low-density lipoprotein cholesterol in short-term studies. This study evaluated whether the evinacumab response was durable in the long term and improved cardiovascular outcome.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS:<\/jats:title>\n                    <jats:p>The OLE ELIPSE HoFH (Open-Label Extension to Evinacumab Lipid Studies in Patients With HoFH) study included newly diagnosed patients and those completing the ELIPSE HoFH trial, on stable lipid-lowering therapy including lipoprotein apheresis but not lomitapide. All patients received evinacumab (15 mg\/kg intravenously) every 4 weeks, with no change in concomitant lipid-lowering treatment during the first 6 months. The primary efficacy end points were the mean absolute and percentage changes in low-density lipoprotein cholesterol from baseline to 6 months. A key secondary end point was cardiovascular event\u2013free survival, which was compared with a control HoFH cohort not treated with evinacumab or lomitapide and matched for age, sex, and lipoprotein apheresis, derived from French Registry of Familial hypercholesterolemia.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS:<\/jats:title>\n                    <jats:p>\n                      Twelve patients, 5 women and 7 men (12\u201357 years), were enrolled in 3 centers in France. At 6 months, the mean low-density lipoprotein cholesterol reduction with evinacumab was 3.7 mmol\/L or 56% (from 6.5 mmol\/L at baseline to 2.8 mmol\/L;\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      &lt;0.0001) and was sustained over the median 3.5-year follow-up. No patients on evinacumab experienced cardiovascular events versus 13 events for 5\/21 (24%) over 4 years in the control cohort (likelihood\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      =0.0267).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS:<\/jats:title>\n                    <jats:p>Real-life, long-term evinacumab adjunctive to lipid-lowering therapy including lipoprotein apheresis led to sustained low-density lipoprotein cholesterol lowering and improved cardiovascular event\u2013free survival of patients with HoFH.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/atvbaha.123.320609","type":"journal-article","created":{"date-parts":[[2024,5,2]],"date-time":"2024-05-02T05:00:41Z","timestamp":1714626041000},"page":"1447-1454","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":32,"title":["Evinacumab and Cardiovascular Outcome in Patients With Homozygous Familial Hypercholesterolemia"],"prefix":"10.1161","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6000-5028","authenticated-orcid":true,"given":"Sophie","family":"B\u00e9liard","sequence":"first","affiliation":[{"name":"Aix Marseille University, APHM, INSERM, INRAE, C2VN, Marseille, France (S.B., R.V.)."},{"name":"APHM, Department of Nutrition, Metabolic Diseases, Endocrinology, La Conception Hospital, Marseille, France (S.B., 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