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Fontan and Fontan Conversion

Fontan and Fontan Conversion

Developed in the early 1970s, the Fontan procedure is used to treat congenital heart defects that have only one side of the heart that is functional. Surgeons at the Walt Disney Pavilion at Florida Hospital for Children use the procedure to divert venous (unoxygenated) blood to the pulmonary arteries without a pumping chamber. The propelling force through the pulmonary circulation is determined by venous pressure. The single functional ventricle serves as a pump to the rest of the body to deliver oxygen and nutrients.

The procedure is normally performed on young patients in two or three stages. The first stage is usually a systemic to pulmonary artery shunt whereby a systemic artery is connected to the pulmonary arteries by way of a Goretex tube graft. At about 6 months of age, the shunt is removed and replaced during a procedure known as the bidirectional Glenn shunt in which the superior vena cava blood flow is directed to the pulmonary arteries after the main pulmonary artery has been detached from the heart. The third stage takes place at about 2 and a half years of age and is known as the Fontan operation. 

Because the body still may not be receiving adequate levels of oxygen, the Fontan operation directs the blood from the inferior vena cava, which carries oxygen-depleted blood from the lower body, to the lungs. As a result, the blood can be reoxygenated in the lungs without having to be pumped out using the pressure in your child’s veins alone. Surgeons use this procedure to direct all the venous blood flow to the pulmonary arteries thereby dividing the venous and arterial circulations. This leaves a single ventricle to supply oxygenated blood to the body and establishes proper oxygen levels.

Bibliography

Mavroudis C, Backer CL, Deal BJ: The total cavopulmonary artery Fontan connection using lateral tunnel and extracardiac techniques. Op Tech Thorac Cardiovasc Surg 1997;2:180-95.

Mavroudis C, Backer CL, Deal BJ, Johnsrude CL: Fontan conversion to cavopulmonary connection and arrhythmia circuit cryoablation. J Thorac Cardiovasc Surg 1998;115(3):547-56

Marcelletti CF, Hanley FL, Mavroudis C, McElhinney DB, Abella RF, Marianeschi SM, Seddio F, Reddy VM, Petrossian E, de la Torre T, Colagrande L, Backer CL, Cipriani A, Iorio FS, Fontan F: Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience. J Thorac Cardiovasc Surg 2000;119(2):340-6.

Backer CL, Deal BJ, Mavroudis C, Franklin WH, Stewart RD: Conversion of the failed Fontan circulation. Cardiol Young 2006;16 (suppl 1):85-91

Mavroudis C, Backer CL, Deal BJ, Stewart RD, Franklin WH, Tsao S, Ward K: Evolving anatomic and electrophysiologic considerations associated with Fontan conversion. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2007;10:136-145

Mavroudis C, Deal BJ, Backer CL, Stewart RD, Franklin WH, Tsao S, Ward KM, deFreitas A: J. Maxwell Chamberlain Memorial Paper for Congenital Heart Surgery. 111 Fontan conversions with arrhythmia surgery: surgical lessons and outcomes. Ann Thorac Surg 2007;84(5):1457-66.

Mavroudis C, Backer CL, Deal BJ: Late reoperations for Fontan patients: State of the art invited review. Eur J Cardiothorac Surg 2008;34:1034-1040

Backer CL, Deal BJ, Kaushal S, Russell HM, Tsao S, Mavroudis C: Extracardiac Versus Intra-Atrial Lateral Tunnel Fontan: Extracardiac is Better. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2011; 14:4-10.

Stewart RD, Pasquali SK, Jacobs JP, Benjamin D, Jaggers J, Mavroudis C, Jacobs ML: Contemporary Fontan Operation: Association of Type of Cavopulmonary Connection with Early Outcome. Ann Thorac Surg 2012; 93:1254-1261.

Mavroudis C: A Tribute to Francis Fontan and Guillermo Kreutzer World J Pediatr Cong Heart Surg 2012; 3:156-158.

Larue M, Gossett J,, Stewart RD, Backer CL, Mavroudis C, Jacobs ML: Plastic bronchitis in patients with Fontan physiology: review of the literature and preliminary experience with Fontan conversion and cardiac transplantation. World J Pediatr Cong Heart Surg 2012; 3: 364-372.

 

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