Cardio-Obstetric Care in a Patient With Tricuspid Atresia and Fenestrated Fontan

Pregnancy in patients with congenital heart disease, particularly those with Fontan physiology, presents significant risks for both the mother and fetus.

We present a 26-year-old woman with a history of tricuspid atresia palliated with a modified fenestrated lateral tunnel Fontan, with a successful pregnancy and delivery despite numerous challenges. She was closely monitored with monthly cardio-obstetrics follow-ups, anticoagulation therapy, echocardiography, and regular obstetric ultrasounds. At 31 weeks of gestation, the patient experienced a massive placental abruption, necessitating an emergent caesarean section. Both the patient and her baby recovered well postpartum owing to meticulous predelivery planning.

The Fontan procedure, which reroutes venous blood flow directly to the pulmonary arteries via passive flow, creates unique challenges during pregnancy because of altered circulatory dynamics. There is a high risk for both thrombotic and bleeding complications as well as preterm delivery, as this case represents. Specialized cardio-obstetrics monitoring with a multidisciplinary approach was essential for optimizing outcomes despite the emergent nature of her delivery.

Although pregnancy may be feasible with expert care, the long-term impact on Fontan function remains uncertain and potentially adverse.