The efficiency of long-term low-density lipoprotein apheresis (LDL-apheresis) treatment on the reduction of lipoprotein(a) [Lp(a)] in 37 patients with familial hypercholesterolemia (FH) was investigated during a mean 5-year follow-up period in the Kyushu LDL-Apheresis Study (KLAS-II). After a single LDL-apheresis treatment, the mean reduction rates of total cholesterol, triglyseride, LDL-cholesterol (LDL-C) and Lp(a) were 64 ± 11 (±SD), 67 ± 16, 72 ± 13, and 68 ± 18%, respectively, and the reduction rates that remained stable during the 5-year follow-up period were comparable to those documented in K-LAS-I. A tendency for a decrease in the mean pretreatment levels of these lipid parameters was also observed. A significant correlation between the rate of decrease in pretreatment Lp(a) with that in LDL-C was statistically reconfirmed (r = 0.690, p < 0.001). Cardiovascular events such as death, myocardial infarction, and newly refractory ischemic symptoms that required either PTCA or CABG occurred in 7 (18%) of our 38 patients after entry into K-LAS, including one patient who died of sudden heart failure before completing K-LAS-II. It was concluded that long-term aggressive LDL-apheresis treatment is effective in the secondary prevention of coronary heart disease in the FH patients who are at higher risk of experiencing future cardiovascular events.