Complex surgical reconstruction including ascending aorta replacement under deep hypothermic circulatory arrest, aortic root enlargement, and valve replacement can be safely performed in patients with homozygous familial hypercholesterolemia and severe calcific aortic disease.
Supports feasibility of complex aortic reconstruction in homozygous FH; hypothesis-generating pending larger studies.
A 61-year-old male with homozygous familial hypercholesterolemia presented with dyspnea and syncope. He had been treated with low-density lipoprotein apheresis for 26 years. Echocardiography and computed tomography showed severe valvular and supravalvular aortic stenosis. Computed tomography and cardiac catheterization revealed a severely calcified narrowed aortic root and an occlusion in the proximal right coronary artery. During surgery, the ascending aorta was replaced under deep hypothermic circulatory arrest without aortic cross-clamping. After that, the aortic root from the annulus to the sino-tubular junction was enlarged with a two-ply bovine pericardial patch. An aortic valve replacement with a 17 mm mechanical valve and coronary artery bypass grafting to the right coronary artery were performed. The patient recovered from the surgery without any cerebrovascular complications.
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Satô et al. (2014) studied this question.
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