Open Access
AJP Rep 2016; 06(01): e145-e147
DOI: 10.1055/s-0036-1581058
Case Report
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Perinatal Management of Pregnancy Complicated by Autosomal Dominant Emery–Dreifuss Muscular Dystrophy

Megumi Sato
1   Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan
,
Hiromitsu Shirasawa
1   Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan
,
Kenichi Makino
1   Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan
,
Hiroshi Miura
1   Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan
,
Wataru Sato
1   Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan
,
Dai Shimizu
1   Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan
,
Naoki Sato
1   Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan
,
Jin Kumagai
1   Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan
,
Akira Sato
1   Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan
,
Yukihiro Terada
1   Department of Obstetrics and Gynecology, Akita University Graduate School of Medicine, Akita, Japan
› Author Affiliations
Further Information

Publication History

05 August 2015

19 February 2016

Publication Date:
31 March 2016 (online)

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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.