Pregnancy and Delivery in Patients With Fontan Circulation

UNLABELLED: The Fontan connection, originally described in 1971, is used to provide palliation for patients with many forms of congenital heart disease that cannot support a biventricular circulation. An increasing number of women who have undergone these connections in childhood are now surviving into adulthood, and some are becoming pregnant. The low flow and fixed cardiac output of a Fontan circulation pose a number of problems during pregnancy. Here, we report a case of a woman who underwent a Fontan procedure at age 7 and experienced significant cardiovascular decline before successfully delivering a viable infant at 33 weeks gestation. In addition, we reviewed the pertinent published data in this area, which suggests that pregnant patients with a Fontan circulation are more likely to face obstetrical, rather than cardiovascular, complications, including preterm labor, intrauterine growth restriction, an increased risk of cesarean section, and the potential need for anticoagulation. The review provides the obstetrician with the information needed to take a prominent role in the appropriate management of this rare, but growing, patient population. TARGET AUDIENCE: Obstetricians & Gynecologists, Family Physicians Leaning Objectives: After completion of this article, the reader will be able to describe the Fontan circulation, describe the importance of the collaborative practice model for patients with Fontan circulation, and identify potential complications in the pregnancy of women with Fontan circulation.