Successful surgical treatment for aortic valve regurgitation and thoracoabdominal aortic aneurysm in a patient with homozygous familial hypercholesterolemia; report of a case]

A 46-year-old woman with homozygous familial hypercholesterolemia was referred due to aortic regurgitation. The patient was introduced selective low density lipoprotein cholesterol(LDL)apheresis 20 years ago. Echocardiogram revealed severe aortic regurgitation, and computed tomography revealed thoracoabdominal aortic aneurysm. We considered 2 staged operations were necessitated. Firstly, aortic valve replacement was performed. Emergent coronary artery bypass grafting was also done because intraoperative myocardial ischemia was strongly suspected from left ventricular hypokinesis. One year later, replacement of thoracoabdominal aorta was performed. Post-operative course was uneventful and the patient was discharged at post-operative day 21. The patients with homozygous familial hypercholesterolemia must be strictly followed up because systemic atherosclerosis frequently exacerbates despite selective LDL apheresis.