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)

Prior treatment

• Patients participating in a clinical trial with an investigational drug within the last 6 months.
• Previous multi-vessel coronary artery bypass grafting (CABG). Patients with single-vessel CABG are not excluded.
• Participation in any interventional clinical trial involving investigational drugs within the last 6 months.
• Previous multi-vessel coronary artery bypass grafting (CABG). Patients with single-vessel CABG are not excluded.

Other illness

• Moderate to severe renal impairment, or end-stage renal disease (ESRD) undergoing kidney transplantation or chronic renal replacement therapy.
• Moderate to severe renal impairment, or end-stage renal disease (ESRD) undergoing kidney transplantation or chronic renal replacement therapy.

Other

• Patients treated outside of Evinacumab approved indication.
• Inability to access adequate retrospective clinical data from medical records.
• Inability or unwillingness to provide informed consent/assent or refusal to participate.
• Pregnancy at the time of the LLT administration.
• Inability to access adequate retrospective clinical data from medical records.
• Inability or unwillingness to provide informed consent/assent or refusal to participate.
• Pregnancy at the time of the LLT administration.

• Willing and able to provide written informed consent form/assent form for the use of retrospective and prospective data.
• Male or female patients aged ≥12 years old at time of enrolment (exception for Italy and France ≥18 years).
• Clinical or genetic diagnosis of Homozygous Familial Hypercholesterolemia (HoFH) according to the consensus statement by Cuchel et al, 2023; EHJ (14).
• Patients who initiated Evinacumab (at the approved dosage and administration) within 24 months before enrolment as add-on to lipid-lowering treatment (with statins, ezetimibe, PCSK9i and/or other agents or treatment) at stable dose for at least 30 days before Evinacumab initiation, as per routine clinical care and no change in dosing is anticipated.
• Availability of a baseline CCTA performed at least 6 months prior or 1 month after Evinacumab initiation and a follow-up CCTA performed 18-24 months after Evinacumab initiation.
• LDL-cholesterol ≥ 140 mg/dl (3.5 mmol/L) despite lipid-lowering treatment.
• Willing and able to provide written informed consent form/assent form for the use of retrospective and prospective data.
• Male or female patients aged ≥12 years old at time of enrolment (exception for Italy and France ≥18 years).
• Clinical or genetic diagnosis of Homozygous Familial Hypercholesterolemia (HoFH) according to the consensus statement by Cuchel et al, 2023; EHJ (14).
• Patients with HoFH on standard lipid-lowering therapy (statins, ezetimibe, PCSK9i and/or other agents or treatment) at stable dose for at least 30 days before a baseline CCTA and with a follow-up CCTA after 18-24 months from the baseline one.
  • Required lipid lowering therapies: statin, ezetimibe and PCSK9 directed therapy (unless discontinuation due to \<15% LDL-cholesterol reduction).
  • Optional additional lipid-lowering therapies:
  • Lipoprotein apheresis, at stable intervals for at least 3 months.
  • Lomitapide, at stable dose for at least 3 months.
• LDL-cholesterol ≥ 140 mg/dl (3.5 mmol/L) despite lipid-lowering treatment.