Progressive Aortic Stenosis in Homozygous Familial Hypercholesterolemia After Liver Transplant

Area of Science:

• Cardiology
• Vascular Biology
• Transplantation

Background:

• Homozygous familial hypercholesterolemia (HoFH) causes early-onset cardiovascular disease, particularly aortic calcifications.
• Standard treatments like diet, medication, and lipoprotein apheresis aim to reduce low-density lipoprotein cholesterol (LDL-C).
• Liver transplantation normalizes lipid levels in HoFH patients but doesn't always halt vascular calcification progression.

Observation:

• Vascular calcifications can progress despite optimal medical therapy and even after liver transplantation.
• Aortic valve stenosis progression has been reported post-transplant, sometimes linked to graft rejection.
• This case details severe aortic valve stenosis progression in an HoFH patient post-liver transplant without rejection.

Findings:

• The patient exhibited progressive severe aortic valve stenosis.
• Lipid levels were normalized post-liver transplant.
• There was no history of liver graft rejection.

Implications:

• Liver transplantation may not prevent the progression of vascular calcification in HoFH.
• Further research is needed to understand the mechanisms driving calcification post-transplant.
• This finding suggests alternative or adjunctive therapies may be necessary for managing cardiovascular risk in HoFH patients post-transplant.