Cardiovascular outcome of pediatric patients with bi-allelic (homozygous) familial hypercholesterolemia before and after initiation of multimodal lipid lowering therapy including lipoprotein apheresis
The American Journal of Cardiology Oct 05, 2020
Taylan C, Driemeyer J, Schmitt CP, et al. - Researchers performed this retrospective analysis to determine cardiovascular outcome among patients (n = 24) with bi-allelic familial hypercholesterolemia initiating chronic lipoprotein apheresis (LA) at a mean age of 8.5 ± 3.1 years. Also, this patient sample was in part prospectively examined with a mean follow-up of 17.2 ± 5.6 years. A mean LDL-C level of 184 mg/dl (4.8 mmol/l), which meant a 75.5% mean reduction, was brought about by multimodal lipid lowering therapy including LA. Following initiation of chronic LA, 16 patients (67%) continued to be clinically stable with merely subclinical findings of atherosclerotic cardiovascular disease (ASCVD), and neither encountered cardiovascular events nor required vascular interventions or surgery. In the light of findings of this study, experts recommended regular evaluation of both the aortic valve, as well as all arteries accessible by ultrasound, in this rare patient population in order to adjust the intensity of multimodal lipid lowering therapy with the objective to avoid ASCVD events as well as aortic surgery.
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