Ladouceur Magalie, Girnius Andrea, Zentner Dominica, Valente Anne Marie, Economy Katherine E, Roos-Hesselink Jolien, Warshak Carri, Partington Sara, Gao Zhiqian, Ollberding Nicholas J, Faust Michelle, Kaemmerer Harald, Akagi Teiji, Grewal Jasmine, Bradley Elisa A, Buber Jonathan, Palumbo Joseph S, Walker Niki, Aboulhosn Jamil, Book Wendy, Pieper Petronella G, Nagdyman Nicole, Cohen Scott, Mulder Barbara J M, Oechslin Erwin, Baumgartner Helmut, Kurdi Wesam, Canobbio Mary M, Veldtman Gruschen
Department of Cardiology, University Hospital of Geneva, Geneva, Switzerland. Department of Cardiology, Adult congenital heart disease unit, Hôpital Européen Georges Pompidou, AP-HP, Paris, France.
Anesthesiology, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.
Eur J Prev Cardiol. 2025 Jul 22. doi: 10.1093/eurjpc/zwaf456.
Pregnancy in women with a Fontan circulation carries increased risk. Given the relative evidence void, pregnancy counseling requires considerable nuance and experience.
This study aimed to identify risk factors for maternal and fetal complications and to contrast risk estimates obtained from existing risk stratification tools, including the modified WHO, CARPREG II, and ZAHARA risk scores.
Pregnant women (>20 weeks of gestation) with a Fontan circulation were retrospectively included from 13 international centers. Univariate and multivariable analyses identified predictors of complications, and the performance of risk stratification tools was assessed.
From 2006 to 2018, 84 women with Fontan physiology had 108 pregnancies, and form the basis of this investigation. Maternal cardiovascular complications occurred in 32 (30%) of all pregnancies including Fontan circulatory failure (17%), supraventricular tachycardia (7.4%), and thromboembolic events (3.7%). No maternal deaths occurred. Premature birth constituted 68% of neonatal complications, with a fetal and neonatal mortality of 13%. Multivariable analysis linked adverse maternal outcomes to pre-pregnancy oxygen saturation (adjusted OR 0.77; 95%CI 0.61-0.96; p=0.02). None of the risk models showed good discriminative ability. The modified WHO classification was the only risk model significantly associated with adverse fetal outcomes.
Pregnancy in women with Fontan circulation poses a significant cardiovascular risk for the mother and a high burden of fetal and neonatal complications. The existing predictive risk stratification models do not discriminate hazard between well-functioning patients with Fontan from those with additional hemodynamic burden. This underscores the necessity for large-scale studies to refine risk stratification.
患有Fontan循环的女性怀孕风险增加。鉴于相关证据不足，孕期咨询需要相当的细微差别和经验。
本研究旨在确定母婴并发症的风险因素，并对比现有风险分层工具（包括改良的世界卫生组织、CARPREG II和ZAHARA风险评分）得出的风险估计值。
回顾性纳入来自13个国际中心的妊娠超过20周且患有Fontan循环的孕妇。单因素和多因素分析确定并发症的预测因素，并评估风险分层工具的性能。
2006年至2018年，84名具有Fontan生理特征的女性共怀孕108次，构成了本研究的基础。在所有妊娠中，32例（30%）发生了母亲心血管并发症，包括Fontan循环衰竭（17%）、室上性心动过速（7.4%）和血栓栓塞事件（3.7%）。未发生母亲死亡。早产占新生儿并发症的68%，胎儿和新生儿死亡率为13%。多因素分析将不良母亲结局与孕前血氧饱和度相关联（调整后的比值比为0.77；95%置信区间为0.61 - 0.96；p = 0.02）。没有一个风险模型显示出良好的判别能力。改良的世界卫生组织分类是唯一与不良胎儿结局显著相关的风险模型。
患有Fontan循环的女性怀孕对母亲构成重大心血管风险，胎儿和新生儿并发症负担也很高。现有的预测性风险分层模型无法区分功能良好的Fontan患者与有额外血流动力学负担的患者之间的风险。这凸显了进行大规模研究以完善风险分层的必要性。
