Pregnancy after Fontan repair of tricuspid atresia

We have described a patient who had a cyanotic congenital heart disease (type Ib tricuspid atresia), with initial palliation accomplished in childhood via a Glenn procedure. In 1985, she had a Fontan repair with the Bjork modification; 3 years later she achieved her first pregnancy at age 27. Maternal Doppler echocardiography in early pregnancy showed good flow through the constructed conduit, with normal left ventricular size and function. Fetal echocardiography at 22 weeks' gestation via two-dimensional, M-mode, pulsed Doppler, and color flow mapping revealed no evidence of fetal cardiac disease. At 25 weeks' gestation recalcitrant preterm labor developed, and the infant was delivered spontaneously. Labor, delivery, and puerperium were uncomplicated, and the newborn (though too premature to survive) was of appropriate weight for gestational age, without evidence of congenital heart disease or other anomalies. We believe this is the first report of pregnancy and spontaneous delivery in a patient who has had Fontan repair of a congenital heart defect.