Aortic valve replacement for the calcified ascending aorta in homozygous familial hypercholesterolemia

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.

Successful surgical management for aortic and mitral stenosis in homozygous familial hypercholesterolemia: a case report

“…Yasuda et al 4 performed endoarterectomy of the ascending aorta under temporary circulatory arrest, followed by aortic valve replacement under aortic cross-clamping; Saito et al 5 did replacement of the ascending aorta under deep hypothermic arrest and aortic valve replacement during rewarming in patients with aortic stenosis and homozygous hypercholesterolemia. We did not use deep hypothermic arrest for the entire period of distal anastomosis of the vascular prosthesis, which enables saving extracorporeal time for core cooling and rewarming.…”

Aortic valve replacement and coronary bypass in a patient with homozygous familial hypercholesterolemia

“…The surgical management of these patients is challenging 4,5 . The atheromatous plaques and calcification of the ascending aorta make aortic canulation and cross clamping hazardous.…”

“…Our patient developed a cerebral infarct presumably due to the cross clamping of the diseased aorta. Methods to avoid the need to cross clamp the aorta have been described 4,5 . Total circulatory arrest to avoid the need to cross clamp the aorta was described by Saito 4 and Yasuda 5 .…”

“…The most lethal manifestation is premature coronary atherosclerosis and aortic valvular and supra valvular stenosis 1,2,3 . Diffuse involvement of the aorta makes the surgery extremely challenging 4,5 We report our experience with the management of a patient with homozygous familial hypercholesterolemia ( HFH).…”

Coronary artery bypass surgery in the second decade of life

“…The atheromatous plaques and calcification of the ascending aorta make surgical management hazardous and challenging. Various techniques like total circulatory arrest and peripheral arterial cannulation have been described to avoid manipulation of the aorta [7]. When ascending aorta and root are heavily calcified replacement of the ascending aorta and the root maybe required.…”