LDL-apheresis and improvement in the coronary atherosclerosis of familial hypercholesterolemia--correlation of computerized quantitative coronary angiography with autopsy findings.

We have evaluated the effects of LDL-aphereses performed over 15 to 62 months, involving both Double Filtration Plasmapheresis (DFPP) and LDL Adsorbent Plasmapheresis (LAPP), for 5 patients with familial hypercholesterolemia (FH) (1; homozygous, and 4; heterozygous) using computer image analysis by coronary angiography, (CAG). Results by CAG showed that in homozygous FH, 9 (75%) of 12 segments demonstrated no progression, 2 (16.7%) segments showed regression, and only 1 (8.3%) segment showed progression. In heterozygous FH, 27 (81.8%) of 33 segments showed progression, and 6 (18.2%) segments showed regression. Aorto coronary bypass was beneficial with obtained patency in 13 (93%) of the 14 grafts. We also performed an autopsy on one patient, with heterozygous FH who died suddenly probably due to fibrillation. The patient had received long-term LDL apheresis for 6 years and 7 months and had shown angiographic regression. The pathological findings showed no typical or new atheroma, significant cicatrization in the thickened intima and an eccentric thickened wall lesion. The serial angiographic findings together with the pathological findings very clearly support the use of LDL-apheresis for producing "true" regression in coronary atherosclerosis in FH.