title: Lipoprotein apheresis is essential for managing pregnancies in patients with homozygous familial hypercholesterolemia: Seven case series and discussion.
abstract: For patients with homozygous familial hypercholesterolemia (HoFH), atherogenic lipoprotein changes and increased stress on cardiovascular system during pregnancy may pose substantial risk for both the mother and her fetus. Although lipoprotein apheresis (LA) is reported as the most effective therapy to control LDL-C levels during pregnancy in HoFH patients, only case reports have been published, and there is no guidance for management.
  
  
  
  We report twelve pregnancies and ten deliveries in seven patients with HoFH, and compare the clinical outcomes between patients who received LA during pregnancy and those who did not.
  
  
  
  One patient who refused LA during pregnancy died from acute myocardial infarction after delivery. Another patient whose adherence to LA was poor also died of myocardial infarction during pregnancy. One patient who initiated LA at the age of 18 had to discontinue LA due to severe symptoms of angina pectoris during pregnancy. Another had symptoms of nausea, hypotension, and bradycardia with increased levels of serum bradykinin during a dextran sulfate cellulose absorption-based LA procedure. Although two of the other three patients had already had coronary artery disease by the time of pregnancy, early initiation of LA from childhood and good adherence to it during pregnancy resulted in the delivery of healthy infants without adverse effects.
  
  
  
  LA is essential for managing pregnancy safely in patients with HoFH. Increasing numbers of documented cases, including ours, will be helpful to guide future therapeutic decisions.
authors.affiliation: Department of Molecular Innovation in Lipidology, National Cerebral and Cardiovascular Center Research Institute, Osaka, Japan. Electronic address: enustasam@ncvc.go.jp.
authors.forename: Masatsune
authors.initials: M
authors.lastname: Ogura
authors.affiliation: Division of Endocrinology and Metabolism, National Cerebral and Cardiovascular Center Hospital, Osaka, Japan.
authors.forename: Hisashi
authors.initials: H
authors.lastname: Makino
authors.affiliation: Department of Perinatology, National Cerebral and Cardiovascular Center Hospital, Osaka, Japan.
authors.forename: Chizuko
authors.initials: C
authors.lastname: Kamiya
authors.affiliation: Department of Perinatology, National Cerebral and Cardiovascular Center Hospital, Osaka, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Yoshimatsu
authors.affiliation: Department of Medicine, Central Manchester University Hospital NHS Foundation Trust, Manchester, United Kingdom.
authors.forename: Handrean
authors.initials: H
authors.lastname: Soran
authors.affiliation: Department of Medicine, Central Manchester University Hospital NHS Foundation Trust, Manchester, United Kingdom.
authors.forename: Ruth
authors.initials: R
authors.lastname: Eatough
authors.affiliation: Department of Obstetrics and Gynecology, "Sapienza" University of Rome, Roma, Italy.
authors.forename: Giuseppina
authors.initials: G
authors.lastname: Perrone
authors.affiliation: Department of Molecular Innovation in Lipidology, National Cerebral and Cardiovascular Center Research Institute, Osaka, Japan.
authors.forename: Mariko
authors.initials: M
authors.lastname: Harada-Shiba
authors.affiliation: Departments of Immunohematology and Transfusion Medicine and Department of Molecular Medicine - Lipid Clinic and Atherosclerosis Prevention Centre, "Sapienza" University of Rome, Roma, Italy.
authors.forename: Claudia
authors.initials: C
authors.lastname: Stefanutti
mesh_terms: D000293:Adolescent; D000069059:Atorvastatin / Q000008:administration & dosage; D001781:Blood Component Removal / Q000379:methods*; D001920:Bradykinin / Q000737:chemistry; D002648:Child; D002675:Child, Preschool; D003657:Decision Making; D005260:Female; D006720:Homozygote; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000235:genetics*; D007223:Infant; D008074:Lipoproteins / Q000097:blood*; D009203:Myocardial Infarction / Q000150:complications* / Q000401:mortality*; D010349:Patient Compliance; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000378:metabolism / Q000628:therapy*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D008074:Lipoproteins; D000069059:Atorvastatin; D001920:Bradykinin
keywords: Aortic valvular disease; Coronary artery disease; Homozygous familial hypercholesterolemia; Lipoprotein apheresis; Pregnancy