Pathological and angiographic regression of coronary atherosclerosis by LDL-apheresis in a patient with familial hypercholesterolemia

Abstract

We present the case of a 42-year-old male with familial hypercholesterolemia (FH) who had received long-term low-density lipoprotein (LDL)-apheresis before death occurred, presumably from an arrhythmia. He had been treated with double filtration plasmapheresis (DFPP) for 4 years and selective LDL adsorbent plasmapheresis (LAPP) for 2 years and 7 months. During the period of treatment (6 years and 7 months) he had received a total of 129 sessions of LDL-apheresis. Serum total cholesterol of the patient before the treatment was 638 mg/dl and during the treatment, the time-averaged values ranged from 336 mg/dl to 411 mg/dl for the first 4 years (with DFPP) and from 257 mg/dl to 364 mg/dl for the sequential 2 years and 7 months (with LAPP). Coronary angiograms were analysed for 13 segments of the coronary arteries using a digitized processing system. Analysis documented regression by identifying a reduction in percent stenosis from 34% to 20% in the proximal left circumflex artery (LCX), from 78% to 61% in the proximal right coronary artery (RCA), and from 92% to 72% in the middle RCA. In the other 10 segments analysed no significant regression and no progression were observed. The autopsy findings of the step-wise serial sections of the native coronary arteries did not record the formation of new and/or typical atheroma. In addition, a thickened intima, and an eccentric thickened wall lesion rich in collagen fiber were observed, although an accumulation of foam cells in the thickened wall lesions was found in some segments. This observation suggested scarring of the atheromatous plaque. We confirmed that LDL-apheresis performed over a period of 6 years and 7 months induced angiographic regression-,of coronary atherosclerosis in the patient with FH, and found that most of the atherosclerotic lesions were changed pathologically into sclerotic lesions rich in collagen fiber.