Four Successful Pregnancies After Fontan Palliation
Four Successful Pregnancies After Fontan Palliation
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.
The Fontan operation was introduced as a palliative procedure for patients with tricuspid atresia and has since been used extensively to provide palliation for patients with several forms of congenital heart disease that cannot support biventricular circulation. The vena cava flow is directed to the pulmonary arteries without passing through the ventricle. It was initially described in 1971. Since then, the Fontan procedure has evolved over the years, most notably with the substitution of right atrial-to-pulmonary artery anastomosis with cavopulmonary connections. This progress resulted in significantly improved late outcomes; an increasing number of women who have undergone these connections in childhood are now surviving into adulthood, and some of them are becoming pregnant. Pregnancy after Fontan procedure has one of the highest risks in women with congenital heart disease and therefore requires careful prior consideration. Strict perinatal care is mandatory in these women. Reports on pregnancy in women with a prior Fontan connection are scarce. Here we describe four successful pregnancies in a woman after Fontan palliation.
Abstract and Introduction
Abstract
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.
Introduction
The Fontan operation was introduced as a palliative procedure for patients with tricuspid atresia and has since been used extensively to provide palliation for patients with several forms of congenital heart disease that cannot support biventricular circulation. The vena cava flow is directed to the pulmonary arteries without passing through the ventricle. It was initially described in 1971. Since then, the Fontan procedure has evolved over the years, most notably with the substitution of right atrial-to-pulmonary artery anastomosis with cavopulmonary connections. This progress resulted in significantly improved late outcomes; an increasing number of women who have undergone these connections in childhood are now surviving into adulthood, and some of them are becoming pregnant. Pregnancy after Fontan procedure has one of the highest risks in women with congenital heart disease and therefore requires careful prior consideration. Strict perinatal care is mandatory in these women. Reports on pregnancy in women with a prior Fontan connection are scarce. Here we describe four successful pregnancies in a woman after Fontan palliation.
