Katsurahgi Shinji, Kamiya Chizuko, Yamanaka Kaoru, Neki Reiko, Miyoshi Takekazu, Iwanaga Naoko, Horiuchi Chinami, Tanaka Hiroaki, Yoshimatsu Jun, Niwa Koichiro, Takagi Yaemi, Ogo Takeshi, Nakanishi Norifumi, Ikeda Tomoaki
Department of Obstetrics and Gynecology, Sakakibara Heart Institute, Japan; Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, Japan.
Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, Japan.
Taiwan J Obstet Gynecol. 2019 Mar;58(2):183-187. doi: 10.1016/j.tjog.2019.01.002.
The goal of the study was to clarify the risk factors for pregnancy complicated with Eisenmenger syndrome (ES).
A retrospective study was performed in 15 patients with ES who were managed throughout pregnancy at one institution from 1982 to 2013. Cases associated with congenital heart diseases other than atrial septal defect (ASD), ventricular septal defect (VSD), and patent ductus arteriosus (PDA) were excluded.
The congenital heart diseases in ES included ASD (n = 3), VSD (n = 9), and PDA (n = 3). Ten women chose termination and 5 continued with their pregnancies. In the 5 continuation cases (PDA 1, VSD 4), worsening of cyanosis, exertional fatigue and dyspnea appeared between 25 and 30 weeks gestation and cesarean section was performed at 30 (28-33) weeks. LVEF, PaO, and SpO decreased and heart rate increased significantly from before pregnancy to 25-30 weeks gestation. From before to during the pregnancy, there were no significant changes in mean PABP or pulmonary vascular resistance (PVR) in four cases with data (582-592, 885 to 868, 1280 to 1291, 1476-1522 dyn × s/cm). PVR at conception had a negative relationship with delivery weeks. NYHA classes before, during and 1 year after pregnancy were II, III and II. In one recent case, epoprostenol and tadalafil were administered during pregnancy.
Pregnancy with ES has a high risk due to hypooxygenation, cyanosis, and cardiac failure, which can appear as common complications as early as the 2nd trimester. Early interventions with meticulous care are required for these complications during pregnancy and delivery.
本研究的目的是阐明妊娠合并艾森曼格综合征（ES）的危险因素。
对1982年至2013年在一家机构接受全程孕期管理的15例ES患者进行回顾性研究。排除合并除房间隔缺损（ASD）、室间隔缺损（VSD）和动脉导管未闭（PDA）以外的先天性心脏病的病例。
ES中的先天性心脏病包括ASD（n = 3）、VSD（n = 9）和PDA（n = 3）。10名女性选择终止妊娠，5名继续妊娠。在5例继续妊娠的病例中（PDA 1例，VSD 4例），妊娠25至30周出现紫绀加重、劳力性疲劳和呼吸困难，并在30（28 - 33）周进行剖宫产。从妊娠前到妊娠25 - 30周，左心室射血分数（LVEF）、动脉血氧分压（PaO）和脉搏血氧饱和度（SpO）下降，心率显著增加。在有数据的4例患者中（582 - 592、885至868、1280至1291、1476 - 1522 dyn×s/cm），妊娠前后平均肺动脉压（PABP）或肺血管阻力（PVR）无显著变化。受孕时的PVR与分娩孕周呈负相关。妊娠前、妊娠期间及产后1年的纽约心脏协会（NYHA）心功能分级分别为Ⅱ级、Ⅲ级和Ⅱ级。在最近的1例病例中，妊娠期间使用了依前列醇和他达拉非。
ES患者妊娠因低氧血症、紫绀和心力衰竭而具有高风险，这些并发症早在孕中期就可能出现。孕期和分娩期间需要对这些并发症进行细致护理的早期干预。
