THE EFFECT OF LDL APHERESIS ON PROGRESSION OF CORONARY-ARTERY-DISEASEIN PATIENTS WITH FAMILIAL HYPERCHOLESTEROLEMIA - RESULTS OF A MULTICENTER LDL APHERESIS STUDY

This study investigated the effect of extracorporal lipid-lowering the rapy by low-density lipoprotein (LDL) apheresis on coronary artery dis ease in a population characterized by early development and rapid prog ression of atherosclerosis. We treated 32 patients aged between 15 and 63 years with drug-refractory familial hypercholesterolemia, treated once a week by immune-specific LDL apheresis for 3 years in a controll ed prospective and non-randomized trial; 25 patients (14 females and 1 1 males) completed the study. Noninvasive data were obtained by physic al examination, 12-lead ECG and exercise testing. Invasive cardiologic al data were obtained by cardiac catheterization according to a standa rdized protocol in four cardiological centers. Left ventricular ejecti on fraction was calculated using planimetry. Coronary stenoses were me asured quantitatively in 23 defined coronary segments by a panel of fo ur investigators with an electronic digital caliper. In addition, over all coronary atherosclerosis was visually qualified. Final decisions o n a classification into one of three groups (regression, no change, pr ogression) of coronary atherosclerosis were based on panel consensus. Six cardiac events occurred throughout the study: percutaneous translu minal coronary angioplasty in one patient, coronary bypass grafting in three and two deaths. Statistical analysis of exercise testing yielde d no significant change for maximum power and work capacity during the study period. Hemodynamic data revealed no significant change; mean e jection fraction was calculated as 65.8 +/- 15.9% at study entry and 6 7.0 +/- 12.7% at completion. Quantitative measurement of 111 circumscr ibed coronary stenoses showed a mean stenosis degree of 45 +/- 26% at entry cineangio-film and 43 +/- 22% at final cineangio-film demonstrat ing no significant change. Eight localized stenoses showed a regressio n of more than 10% and 11 stenoses a progression of more than 10%. Pan el consensus decision for overall coronary atherosclerosis resulted in regression in no patients, no change in 16, questionable progression in 3, definite progression in 5, and undecided in one. We conclude tha t specific LDL-apheresis therapy can be used effectively for primary a nd secondary prevention of coronary artery disease in patients with fa milial hypercholesterolemia. Its beneficial effect was the prevention of further progression of coronary artery disease in the majority of t he study population.