Drenthen W, Pieper P G, Roos-Hesselink J W, van Lottum W A, Voors A A, Mulder B J M, van Dijk A P J, Vliegen H W, Sollie K M, Moons P, Ebels T, van Veldhuisen D J
Department of Cardiology, University Medical Center Groningen, University of Groningen, PO Box 30.001, 9700 RB Groningen, The Netherlands.
Heart. 2006 Sep;92(9):1290-4. doi: 10.1136/hrt.2005.085407. Epub 2006 Jan 31.
To evaluate the outcome of pregnancy in women after Fontan palliation and to assess the occurrence of infertility and menstrual cycle disorders.
Two congenital heart disease registries were used to investigate 38 female patients who had undergone Fontan palliation (aged 18-45 years): atriopulmonary anastomosis (n = 23), atrioventricular connection (n = 5) and total cavopulmonary connection (n = 10).
Six women had 10 pregnancies, including five miscarriages (50%) and one aborted ectopic pregnancy. During the remaining four live-birth pregnancies clinically significant complications were encountered: New York Heart Association class deterioration; atrial fibrillation; gestational hypertension; premature rupture of membranes; premature delivery; fetal growth retardation and neonatal death. Four of seven women who had attempted to become pregnant reported female infertility: non-specified secondary infertility (n = 2), uterus bicornis (n = 1) and related to endometriosis (n = 1). Moreover, several important menstrual cycle disorders were documented. In particular, the incidence of primary amenorrhoea was high (n = 15, 40%), which resulted in a significant increase in age at menarche (14.6 (SD 2.1) years, p < 0.0001, compared with the general population).
Women can successfully complete pregnancy after adequate Fontan palliation without important long-term sequelae, although it is often complicated by clinically significant (non-)cardiac events. In addition, subfertility or infertility and menstrual disorders were common.
评估接受Fontan姑息手术的女性的妊娠结局，并评估不孕症和月经周期紊乱的发生率。
使用两个先天性心脏病登记处对38例接受Fontan姑息手术的女性患者（年龄18 - 45岁）进行调查：心房肺吻合术（n = 23）、房室连接术（n = 5）和全腔肺连接术（n = 10）。
6名女性有10次妊娠，包括5次流产（50%）和1次异位妊娠流产。在其余4次活产妊娠中，出现了具有临床意义的并发症：纽约心脏协会心功能分级恶化；心房颤动；妊娠期高血压；胎膜早破；早产；胎儿生长受限和新生儿死亡。7名尝试怀孕的女性中有4名报告存在女性不孕症：未明确的继发性不孕症（n = 2）、双子宫（n = 1）和与子宫内膜异位症相关（n = 1）。此外，记录了几种重要的月经周期紊乱情况。特别是，原发性闭经的发生率很高（n = 15，40%），这导致初潮年龄显著增加（14.6（标准差2.1）岁，与一般人群相比，p < 0.0001）。
女性在接受充分的Fontan姑息手术后可以成功完成妊娠，且无重要的长期后遗症，尽管妊娠常伴有具有临床意义的（非）心脏事件。此外，生育力低下或不育以及月经紊乱很常见。
