Abstract
Homozygous familial hypercholesterolemia (HoFH; Fredrickson IIa) is a rare autosomal dominant hereditary disease associated with increased low-density lipoprotein cholesterol. We hereby report anesthetic challenges in a rare case of HoFH having severe progressive aortic stenosis, mitral regurgitation, diffuse aortic and carotid vessel involvement, and severe left ventricular dysfunction. The patient underwent a Konno aortoventriculoplasty with mechanical prosthesis along with mitral valve repair.
