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Aortic valve replacement for the calcified ascending aorta in homozygous familial hypercholesterolemia

T Yasuda1, M Kawasuji, N Sakakibara

  • 1Department of Surgery (I), Kanazawa University School of Medicine, Takaramachi 13-1, 920-8641, Kanazawa, Japan. yasuda@sf.m.kanazawa-u.ac.jp

Insights

Familial hypercholesterolemia can lead to severe aortic calcification and coronary artery stenosis. Surgical intervention, including aortic valve replacement and bypass grafting with hypothermic circulatory arrest, is a preferred method for managing these complex cardiovascular conditions.

Area of Science:

  • Cardiology
  • Cardiovascular Surgery
  • Genetics

Background:

  • Familial hypercholesterolemia (FH) is a genetic disorder characterized by extremely high levels of low-density lipoprotein cholesterol.
  • Homozygous FH (HoFH) presents with severe, premature atherosclerotic disease, including coronary artery and aortic valve involvement.

Observation:

  • A 72-year-old female patient with diagnosed HoFH presented with chest discomfort.
  • Imaging revealed severe aortic root calcification and high-grade stenosis of the proximal right coronary artery.

Findings:

  • The patient underwent aortic valve replacement concurrently with coronary artery bypass grafting.
  • The surgical procedure utilized temporary hypothermic circulatory arrest.

Implications:

  • This case highlights the critical cardiovascular complications associated with HoFH.
  • Temporary hypothermic circulatory arrest is demonstrated as a viable and preferred surgical approach for complex aortic calcification and coronary artery disease in FH patients.

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