The effect of LDL apheresis on progression of coronary artery disease in patients with familial hypercholesterolemia

Abstract

This study investigated the effect of extracorporal lipid-lowering therapy by low-density lipoprotein (LDL) apheresis on coronary artery disease in a population characterized by early development and rapid progression of atherosclerosis. We treated 32 patients aged between 15 and 63 years with drug-refractory familial hypercholesterolemia, treated once a week by immuno-specific LDL apheresis for 3 years in a controlled prospective and non-randomized trial; 25 patients (14 females and 11 males) completed the study. Noninvasive data were obtained by physical examination, 12-lead ECG and exercise testing. Invasive cardiological data were obtained by cardiac catheterization according to a standardized protocol in four cardiological centers. Left ventricular ejection fraction was calculated using planimetry. Coronary stenoses were measured quantitatively in 23 defined coronary segments by a panel of four investigators with an electronic digital caliper. In addition, overall coronary atherosclerosis was visually qualified. Final decisions on a classification into one of three groups (regression, no change, progression) of coronary atherosclerosis were based on panel consensus. Six cardiac events occurred throughout the study: percutaneous transluminal coronary angioplasty in one patient, coronary bypass grafting in three and two deaths. Statistical analysis of exercise testing yielded no significant change for maximum power and work capacity during the study period. Hemodynamic data revealed no significant change; mean ejection fraction was calculated as 65.8 +/- 15.9% at study entry and 67.0 +/- 12.7% at completion. Quantitative measurement of 111 circumscribed coronary stenoses showed a mean stenosis degree of 45 +/- 26% at entry cineangio-film and 43 +/- 22% at final cineangio-film demonstrating no significant change.(ABSTRACT TRUNCATED AT 250 WORDS)

Ten-year Follow-up of Familial Hypercholesterolemia Patients After Intensive Cholesterol-lowering Therapy

“…Previous reports have suggested that complete prevention of CHD was limited to cases administered LDL-apheresis early in the course. 23,24) However, the unchanged CSI and LVEF suggest that long-term combination therapy results in a satisfactory outcome with additional coronary angioplasty. A pathology report showed scarring of atheromatous plaque and formation of a thick fibrous cap after 7 years of LDL apheresis in a FH patient.…”

Early onset of coronary heart disease in a young woman with familial hypercholesterolemia: coronary findings and effect of short term treatment with high dose atorvastatin

“…Therapy with statins is usually recommended [13], both high dose atorvastatin and rosuvastatin [14]. For more severe cases, LDL apheresis is often required [15], since it was shown to be able to stop the progression of coronary atherosclerosis [16].…”

Management of Familial Hypercholesterolemias in adult patients: Recommendations from the National Lipid Association Expert Panel on Familial Hypercholesterolemia

“…Several clinical trials have confirmed the benefits of LDL apheresis for prevention and slowing the progression of cardiovascular disease, 50,[56][57][58][59][60][61][62] and for other cardiovascular effects such as improvements in endothelial function, 63 coronary vasodilation, 64 microvascular flow, 65 and myocardial perfusion. 66 LDL apheresis has also been shown to promote regression of xanthomas 50 and modify a number of markers associated with vascular disease, including lipoprotein (a), an atherothrombotic marker.…”