Lipoprotein Apheresis for Familial Hypercholesterolemia
Lipoprotein Apheresis for Familial Hypercholesterolemia
Lp apheresis was originally introduced to treat patients with homozygous FH, and almost 40 years later, this remains the main indication. However, its use has now widened to include patients with progressive CVD due to heterozygous FH refractory to statins or to raised levels of Lp(a). Various apheresis methods are currently used to remove LDL and Lp(a) from whole blood or plasma, with little to choose between them in terms of efficacy and safety. There is evidence that weekly or biweekly Lp apheresis combined with lipid-lowering drug therapy prolongs the life expectancy of homozygotes and reduces cardiovascular events in heterozygotes. There are also some data suggesting that Lp apheresis arrests progression of CHD and may reduce events in patients with a raised Lp(a), but a randomized trial is needed to settle this issue. In view of the difficulty of obtaining randomized, controlled trial data, it is imperative to set up registries of all patients undergoing Lp apheresis, so as to record observational data of its effects. Algorithms for monitoring progress of CVD in FH patients are proposed using noninvasive procedures, such as carotid ultrasound and echocardiography of the aortic root. Lastly, Lp apheresis provides the only safe means of treating FH homozygotes during pregnancy.
Conclusion
Lp apheresis was originally introduced to treat patients with homozygous FH, and almost 40 years later, this remains the main indication. However, its use has now widened to include patients with progressive CVD due to heterozygous FH refractory to statins or to raised levels of Lp(a). Various apheresis methods are currently used to remove LDL and Lp(a) from whole blood or plasma, with little to choose between them in terms of efficacy and safety. There is evidence that weekly or biweekly Lp apheresis combined with lipid-lowering drug therapy prolongs the life expectancy of homozygotes and reduces cardiovascular events in heterozygotes. There are also some data suggesting that Lp apheresis arrests progression of CHD and may reduce events in patients with a raised Lp(a), but a randomized trial is needed to settle this issue. In view of the difficulty of obtaining randomized, controlled trial data, it is imperative to set up registries of all patients undergoing Lp apheresis, so as to record observational data of its effects. Algorithms for monitoring progress of CVD in FH patients are proposed using noninvasive procedures, such as carotid ultrasound and echocardiography of the aortic root. Lastly, Lp apheresis provides the only safe means of treating FH homozygotes during pregnancy.
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