Introduction: A Fontan operation is performed to provide palliation for patients with many forms of highly complex congenital heart disease that cannot support a biventricular circulation. Increasing numbers of women who have undergone these connections in childhood are now reaching their childbearing years, and some are becoming pregnant. The low flow and fixed cardiac output of a Fontan circulation poses several problems during pregnancy.
Case presentation: We report the case of four successful pregnancies in a 31-year-old Tunisian woman with congenital tricuspid atresia after Fontan operation. Her pregnancies resulted in delivery of four healthy neonates. Her clinical status remained unchanged.
Conclusions: This case suggests that patients after adequate Fontan palliation could complete pregnancy without long-term cardiac sequelae. Intensive care should be provided with specialists, including a neonatologist, anesthesiologist and cardiologist.