Off-pump coronary bypass in a child with familial hypercholesterolemia: premature atherosclerosis of the ascending aorta.

Familial hypercholesterolemia is an autosomal-dominant disorder in which mutations in the low-density lipoprotein (LDL) receptor gene cause high levels of LDL and premature coronary artery disease in childhood (1). Extra coronary atherosclerotic lesions, including calcification of the aortic valve and root, are present in those patients. Atherosclerotic thickening and obstruction of the ascending aorta have also been documented in adult patients with familial hypercholesterolemia (2). When atherosclerosis involves the ascending aorta, coronary artery bypass grafting (CABG) becomes a high-risk procedure that can result in cerebrovascular complications. Coronary artery bypass grafting with the “no touch” aorta technique is preferred to avoid that devastating complication. However, it is unusual to apply this technique in pediatric patients who undergo CABG.