title: Ehlers-Danlos type IV in pregnancy with a history of myocardial infarction.
abstract: Ehlers-Danlos syndrome (EDS) type IV is an autosomal dominantly inherited connective tissue disorder caused by abnormal type III collagen resulting from heterogenous mutations of the type III procollagen gene (COL3A1). The maternal mortality rate per pregnancy in EDS type IV has been reported as 11.5% to 25%. A 30-year-old Japanese primiparous woman, with a brother who had suffered a bowel rupture due to EDS type IV, became pregnant. She also suffered from myocardial infarction due to coronary artery dissections at 24 years old, and underwent coronary artery bypass grafting. Due to uncontrollable uterine contractions, beta 2-stimulants were administered during 18 to 29 weeks of gestation. Therefore, we performed a cesarean section at 29 weeks of gestation to prevent uterine rupture. She and her baby were discharged without any complications. It was revealed that she had the same mutation as her brother, Gly220Trp, in the (Gly-X-Y)n repeat of the triple-helical domain of COL3A1.
authors.affiliation: Department of Obstetrics and Gynecology, Jichi Medical University School of Medicine, Shimotsuke-shi, Japan. okuchi@jichi.ac.jp
authors.forename: Akihide
authors.initials: A
authors.lastname: Ohkuchi
authors.forename: Shigeki
authors.initials: S
authors.lastname: Matsubara
authors.forename: Kayoko
authors.initials: K
authors.lastname: Takahashi
authors.forename: Soichiro
authors.initials: S
authors.lastname: Inoue
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Saito
authors.forename: Takeshi
authors.initials: T
authors.lastname: Mitsuhashi
authors.forename: Takanori
authors.initials: T
authors.lastname: Yamagata
authors.forename: Naoto
authors.initials: N
authors.lastname: Takahashi
authors.forename: Atsushi
authors.initials: A
authors.lastname: Watanabe
authors.forename: Takashi
authors.initials: T
authors.lastname: Shimada
authors.forename: Mitsuaki
authors.initials: M
authors.lastname: Suzuki
mesh_terms: D000328:Adult; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000401:mortality; D005260:Female; D006801:Humans; D008428:Maternal Mortality; D009203:Myocardial Infarction / Q000150:complications*; D011247:Pregnancy; D011248:Pregnancy Complications / Q000401:mortality; D014597:Uterine Rupture / Q000517:prevention & control