title: Perinatal Management of Pregnancy Complicated by Autosomal Dominant Emery-Dreifuss Muscular Dystrophy.
abstract: Introduction Autosomal dominant Emery-Dreifuss muscular dystrophy (AD-EDMD) is rare compared with other forms of muscular dystrophy and is characterized by cardiac conduction defects. Here, we present the case of a patient diagnosed with AD-EDMD during the first trimester of pregnancy who developed acute preeclampsia and subsequently, congestive heart failure (CHF) following cesarean section. Case A 36-year-old, gravida 0 para 0 woman was diagnosed with AD-EDMD by genetic testing during the first trimester of pregnancy, and she suddenly developed preeclampsia and partial HELLP (hemolytic anemia, elevated liver enzymes, and low platelets) syndrome at 33 weeks of gestation. The patient subsequently developed CHF following cesarean section. Conclusion CHF can occur as a direct result of the cardiac defects arising due to EDMD, and therefore, careful prenatal and postpartum management is recommended for such cases.
authors.affiliation: Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan.
authors.forename: Megumi
authors.initials: M
authors.lastname: Sato
authors.affiliation: Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan.
authors.forename: Hiromitsu
authors.initials: H
authors.lastname: Shirasawa
authors.affiliation: Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Makino
authors.affiliation: Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Miura
authors.affiliation: Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan.
authors.forename: Wataru
authors.initials: W
authors.lastname: Sato
authors.affiliation: Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan.
authors.forename: Dai
authors.initials: D
authors.lastname: Shimizu
authors.affiliation: Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Sato
authors.affiliation: Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan.
authors.forename: Jin
authors.initials: J
authors.lastname: Kumagai
authors.affiliation: Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Sato
authors.affiliation: Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan.
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Terada
keywords: Emeri–Dreifuss muscular dystrophy; HELLP syndrome; congestive heart failure; preeclampsia