Cardiovascular Division, Department of Internal Medicine, Washington University School of Medicine in St. Louis, St. Louis, MO.
Department of Obstetrics and Gynecology, Washington University School of Medicine in St. Louis, St. Louis, MO.
Am J Obstet Gynecol MFM. 2021 Jan;3(1):100257. doi: 10.1016/j.ajogmf.2020.100257. Epub 2020 Oct 17.
The Fontan operation was first performed in 1968 and is a palliative procedure for children born with single ventricle forms of congenital heart disease. Today, 70,000 patients worldwide have Fontan circulation today, half of them women, and with an expected 30-year survival of >80%, this population is expected to double in the next 20 years. The Fontan operation surgically redirects systemic venous blood return directly to the pulmonary circulation, bypassing the single ventricle. This abnormal anatomy results in significant challenges for the cardiovascular system and is marked by a sustained, abnormally elevated systemic venous pressure combined with decreased cardiac output. As more women with Fontan circulation reach childbearing age, understanding the unique risks of pregnancy to the mother and fetus and how to best provide clinical care for these women during pregnancy is imperative. However, there are limited clinical data to guide counseling and management in this population. This expert review offers an analysis of the literature about Fontan circulation during pregnancy and describes our center's current multidisciplinary approach to care for these women in the preconception, antepartum, intrapartum, and postpartum periods.
Fontan 手术于 1968 年首次实施，是一种针对患有单心室先天性心脏病的儿童的姑息性手术。如今，全球有 7 万名患者存在 Fontan 循环，其中一半是女性，预计 30 年生存率>80%，在未来 20 年内，这一人群预计将增加一倍。Fontan 手术通过手术将体循环静脉血直接重新导向肺循环，绕过单心室。这种异常的解剖结构给心血管系统带来了重大挑战，其特征是持续的、异常升高的体循环静脉压和心输出量降低。随着越来越多的 Fontan 循环女性达到生育年龄，了解妊娠对母亲和胎儿的独特风险以及如何在妊娠期间为这些女性提供最佳临床护理至关重要。然而，目前只有有限的临床数据可用于指导该人群的咨询和管理。本专家综述分析了 Fontan 循环在妊娠期间的文献，并描述了我们中心目前在孕前、产前、产时和产后阶段为这些女性提供的多学科护理方法。
