114 - Central venous catheters for lipid apheresis: First experience in young children with homozygous familial hypercholesterolemia (ID 203)
Abstract
Background and Aims
Homozygous familial hypercholesterolemia (HoFH) is an autosomal-dominant genetic disorder leading to extremely increased LDL cholesterol (LDL-C) from birth onwards, resulting in high cardiovascular risk even in early childhood. Lipid-lowering drugs have limited success in hoFH. Lipid apheresis (LA) is highly effective in reducing LDL-C and should be started as early as possible to prevent premature cardiovascular events. However, venous access can be challenging in young children due to small peripheral vessels and severe anxiety. We evaluated central venous catheters (CVCs) as a possible venous access in young children with hoFH.
Methods
CVCs were surgically implanted in 4 children with severe hoFH under 6 years of age. LA was performed weekly or biweekly. For LA, a Therasorb column by Miltenyi Biotec was used with a plasma Adasorb filled with 5% albumin and a volume of 100ml.
Results
LA could dramatically reduce LDL-C >60% in all children. Measurement of intima-media-thickness improved or remained stable. Severe side effects were early catheter infections with sepsis in two children. Accidental self-removal of the CVC was another frequent issue. In two girls, CVCs could be used permanently for 595 and 698 days, respectively. There was no incidence of occlusion, thrombosis or perioperative complications. After 3 years of LA, 3 children are now tolerating repeated peripheral punctures and one boy has an AV-shunt.
Conclusions
In young children with hoFH, permanent CVCs are a viable alternative access technique for LA. The risk of a delayed start of apheresis by far outweighs the risk of severe CVC complications.
