Evaluating the Effect of Adding Evinacumab to Lipid-Lowering Therapy on Coronary Plaque in Patients With Homozygous Familial Hypercholesterolemia Using Coronary CT Angiography

What this Trial Is About

Researchers are evaluating the impact of intensified lipid-lowering therapy including Evinacumab on coronary atherosclerotic plaque burden in patients with Homozygous Familial Hypercholesterolemia HoFH, a rare genetic disorder marked by very high LDL cholesterol from early life and increased risk of early heart disease. This observational study includes both retrospective and prospective data, aiming to understand how Evinacumab affects plaque progression compared to conventional treatments. Participants are divided into two groups based on routine clinical care one receiving intensified therapy with Evinacumab added to stable lipid-lowering treatments, and the other receiving conventional lipid-lowering therapy without Evinacumab. Coronary computed tomography angiography CCTA scans, performed as part of routine care, will assess plaque volume and characteristics over 18-24 months. The study observes changes in non-calcified plaque volume, total plaque burden, high-risk plaque features, and LDL cholesterol levels. Participants will provide data from past and future routine clinical care, including CCTA imaging and laboratory tests. Patient-reported outcomes and adipose tissue characteristics around coronary arteries will also be evaluated. Researchers will track changes over time, focusing on plaque stability or regression, with safety information collected from medical records. The study is expected to last up to 24 months of follow-up with about 52 patients enrolled across multiple European centers.

Assessing the Impact of Intensification of Lipid Lowering Therapy With Guidelines-based Evinacumab Administration on Coronary Plaque Volumes Measured by Coronary Computed Tomography Angiography (CCTA) in Patients With Homozygous Familial Hypercholesterolemia (HoFH)