Patients with the Fontan operation have a unique circulation, with a limited ability to increase cardiac output, and high central venous pressure. They may have diastolic and/or systolic ventricular dysfunction, arrhythmias, thromboembolic complications, or multiorgan dysfunction. All of these factors contribute to reproductive issues, including menstrual irregularities, infertility, recurrent miscarriage, and complications during pregnancy. Although atrial arrhythmias are the most common cardiac complications during pregnancy, patients can develop heart failure and thromboembolic events. Obstetric bleeding, including postpartum hemorrhage, is common. In addition to maternal complications, adverse fetal and neonatal events, such as prematurity and low birthweight, are very common. Counselling about these reproductive issues should begin early. For those who become pregnant, care should be provided by a multidisciplinary cardio-obstetric team familiar with the specific issues and needs of the Fontan population. In this review, we discuss infertility, contraception, and pregnancy in patients with the Fontan operation.
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