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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.
Abstract:
A 72-year-old woman who had been diagnosed as homozygous familial hypercholesterolemia was admitted for chest discomfort. Computed tomography and cardiac catheterization revealed severe calcification of the aortic root and a high grade stenosis of the proximal right coronary artery. Aortic valve replacement concomitant with coronary artery bypass was done using temporary hypothermic circulatory arrest. This is preferred method when dealing the calcified aorta.