Pregnancy in a patient with homozygous familial hypercholesterolemia treated with long-term low-density lipoprotein apheresis.
The pregnancy and delivery of a subject with homozygous familial hypercholesterolemia (FH) and coronary artery disease (CAD) were monitored closely for signs of maternal and fetal distress. Biweekly treatment with low-density lipoprotein (LDL) apheresis using dextran-sulfate cellulose columns was continued throughout the pregnancy, and lipid and lipoprotein levels were evaluated. During the course of the pregnancy and delivery, no signs of maternal coronary insufficiency developed. Serial ultrasonographic measurements of fetal growth indices and the blood flow velocity waveforms (FVWs) of the uterine and umbilical artery did not reveal any sign of fetal growth retardation or insufficiency of the uteroplacental circulation, respectively. During pregnancy, time-averaged concentrations of serum total cholesterol (TC), LDL cholesterol (LDL-C), apolipoprotein (apo) B, and lipoprotein(a) [Lp(a)] showed a gradual decline. Notwithstanding LDL apheresis, a gradual twofold increase of serum triglyceride (TG) levels was found. In the second and third trimester, high-density lipoprotein cholesterol (HDL-C) levels showed a 55% increase that coincided with a 75% reduction in hepatic lipase activity in postheparin plasma, normalizing after parturition. After delivery, lp(a) levels showed an almost twofold increase, which could not be explained by the interruption of LDL apheresis alone, and may be caused by changes in gonadal steroids. Histologic examination of the placenta and the umbilical arteries revealed no atherosclerotic changes, infarctions, or lipid deposits. In general, long-term LDL apheresis in homozygous FH can delay the onset and complications of severe CAD. In case of a pregnancy, LDL apheresis seems feasible and should be continued during the pregnancy to prevent superimposed hyperlipidemia and placental insufficiency.
Kroon
A A
AA
Department of General Internal Medicine, University Hospital Nijmegen, The Netherlands.
Swinkels
D W
DW
van Dongen
P W
PW
Stalenhoef
A F
AF
D000293:Adolescent; D001781:Blood Component Removal; D003327:Coronary Disease / Q000209:etiology; D005260:Female; D006720:Homozygote; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000097:blood / Q000235:genetics* / Q000628:therapy*; D008055:Lipids / Q000097:blood; D008074:Lipoproteins / Q000097:blood; D008077:Lipoproteins, LDL / Q000097:blood*; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular; D016216:Ultrasonography, Prenatal
D008055:Lipids; D008074:Lipoproteins; D008077:Lipoproteins, LDL