Care of a parturient with a Fontan physiology: A case report

Cardiovascular disease in pregnancy is the leading cause of pregnancy-related mortality in developed nations. Congenital heart disease is amongst the most common cardiac conditions that are encountered during pregnancy. Due to improvements in medical and surgical treatments, an increasing number of individuals with congenital heart disease are reaching childbearing age and presenting to labor and delivery units. An example are those born with single-ventricle physiology e.g. tricuspid atresia and hypoplastic left heart syndrome. In these individuals, staged surgical procedures ending with the Fontan procedure are done to improve survival. Pregnant women with uncomplicated Fontan circulations are at significantly increased risk (modified World Health Organization III), and those with Fontan circulation with any complication are at extremely high risk (modified World Health Organization IV) of morbidity and mortality. Parturients with Fontan physiology have unique anesthetic considerations. Peripartum management of these patients is complex and current practice recommendations are not evidence-based. A multidisciplinary team should be established early in pregnancy to care for these patients with a thorough cardiovascular workup being required. Vaginal deliveries with an assisted second stage and epidural analgesia are preferred. Cesarean deliveries are reserved for obstetric indications. Invasive hemodynamic monitoring and central venous access may be warranted in high-risk patients. Maintaining adequate hydration is key to preserving preload in Fontan physiology. Use of vasopressors, inotropes, and diuretics may be required while caring for these patients. Extracorporeal membrane oxygenation is lifesaving when used early. Life-threatening complications can occur in the postpartum period. Therefore, these patients should be monitored in an intensive care unit after delivery. Management of pregnant patients with Fontan physiology requires multidisciplinary collaboration, team work and a knowledge of peripartum considerations. Anesthesiologists must be aware of Fontan physiology and relevant anesthetic considerations so as to provide optimal care to these patients.