Fontan Completion

Book
In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan.
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Excerpt

The Fontan procedure provides a palliative treatment option for pediatric patients with functionally single ventricle congenital heart disease, with an estimated incidence of 0.08 to 0.4 per 1,000 live births. Total right heart bypass was described in 1971 for tricuspid atresia with the Fontan procedure, or the total cavopulmonary connection (TCPC) procedure. There is a relatively recent history of staged procedures created to achieve this result established on the premise that systemic venous return can be surgically connected directly to the pulmonary circulation, as long as the pulmonary pressures are low. This will effectively allow the systemic venous circulation to bypass the heart.

Patients need to be carefully selected to ensure optimal surgical outcomes. Surgical modifications ushered in different eras, and overall, patients are living longer with better outcomes. Morbidity still remains high with multiple extracardiac manifestations of Fontan circulation after the completion of the procedure. Efforts are ongoing to improve understanding of long-term Fontan patients, functional status, and possible innovations in tackling disparities and improving long-term outcomes.

Publication types

  • Study Guide