Pregnancy in a patient with homozygous familial hypercholesterolemia undergoing low-density lipoprotein apheresis by dextran sulfate adsorption

Abstract

Pregnancy and delivery in homozygous familial hypercholesterolemic (HFH) patients is extremely rare. We describe the case of a woman with HFH treated with low-density lipoprotein (LDL) apheresis by dextran sulfate adsorption who became pregnant and reached delivery uneventfully. LDL apheresis was performed biweekly, and lipoprotein analyses in pre-apheresis samples showed progressive increases in triglyceride, total cholesterol, LDL cholesterol, and apolipoprotein (apo) B plasma concentrations. The fractional catabolic rate (FCR) for LDL cholesterol, as estimated by the first-order disappearance constants ( k values) of the recovery of LDL cholesterol concentration to basal values after each apheresis session, increased more than threefold from week-24 to week-4 (labor is considered as time 0). After delivery, basal values were recovered, but normalization was slower for LDL cholesterol than for the other lipidic parameters. High-density lipoprotein (HDL) showed a different pattern: HDL 3 remained stable throughout gestation, whereas HDL 2 cholesterol and apo A-I had a maximum at midgestation, then declined, and finally increased again at late gestation. With the exception of this latter increase of HDL 2, all the other changes in lipoprotein concentrations during pregnancy and postpartum were similar to those found in healthy women. Thus, LDL apheresis does not interfere with physiologic adaptations of lipoprotein metabolism during pregnancy in HFH patients.