Primary Fontan, 1½ Ventricular Repair, and Fontan Conversion Operations in Adults

Abstract

The Fontan operation has undergone many revisions since its introduction by Francis Fontan and Guillermo Kreutzer in 1968. Gone are the venous pathway valves, the right atrial connections to small subpulmonic ventricles, and atriopulmonary connections. The standard therapy for the functionally single-ventricle heart is determined by three distinct time periods. The first is the neonatal management of pulmonary artery flow (by systemic to pulmonary artery shunt, pulmonary artery band, or observation). At approximately 6 months of age, the second stage takes place: systemic to pulmonary artery shunt takedown or pulmonary artery band takedown and a bidirectional Glenn shunt that prepares the single ventricle by volume unloading, resulting in favorable ventricular remodeling. By 2–3 years of age, the Fontan operation is usually performed. Since the introduction of the atrial fenestration, some surgeons have used this obligatory right-to-left shunt in all cases; some surgeons have used it selectively, and others have not used it at all. Most surgeons now use the fenestration selectively, especially in cases with borderline ventricular function, small pulmonary arteries, abundant aortopulmonary artery collaterals, and single-lung physiology.

Figure Credits

• Figs. 11.1–11.14, 11.16–11.19, 11.21: Mavroudis C. Fontan operation and 1½ ventricular repair. In: Mavroudis C, Backer CL, editors. Atlas of Pediatric Cardiac Surgery. London: Springer-Verlag; 2015. p. 225–34. (Reproduced with permission from Springer Nature).

• Fig. 11.15: Backer CL, Mavroudis C. Congenital heart disease. In: Norton JA, Bollinger RR, Chang AE, Lowry SF, Mulvihill SJ, Pass HI, Thompson RW, editors. Surgery: Basic Science and Clinical Evidence. New York: Springer; 2000. p. 1315–32. (Reproduced with permission from Springer Nature).

• Fig. 11.20: Backer CL, Stewart RD, Mavroudis C. The classical and the one-and-a-half ventricular options for surgical repair inpatients with discordant atrioventricular connections. Cardiol Young. 2006;16:91–6. (Reproduced with permission from Cambridge University Press).

• Figs. 11.22, 11.23: Mavroudis C, Backer CL, Kohr LM, Deal BJ, Stinios J, Muster AJ, Wax DF. Bidirectional Glenn shunt in association with congenital heart repairs: the 1½ ventricular repair. Ann Thorac Surg. 1999;68:976–81. (Reproduced with permission of Elsevier).

• Figs. 11.24, 11.26–11.39, 11.41–11.62, 11.66: Mavroudis C. Fontan conversion and arrhythmia surgery. In: Mavroudis C, Backer CL, editors. Atlas of Pediatric Cardiac Surgery. London: Springer-Verlag; 2015. p. 235–54. (Reproduced with permission from Springer Nature).

• Figs. 11.25, 11.67, 11.68: Backer CL, Deal BJ, Mavroudis C, Franklin WH, Stewart RD. Conversion of the failed Fontan circulation. Cardiol Young. 2006;16:85–91. (Reproduced with permission from Cambridge University Press).

• Figs. 11.63–11.65: Mavroudis C, Backer CL, Deal BJ, Johnsrude C, Strasburger J. Total cavopulmonary conversion and maze procedure for patients with failure of the Fontan operation. J Thorac Cardiovasc Surg. 2001;122:863–71. (Reproduced with permission from Elsevier).

• Fig. 11.40: Mavroudis C, Backer CL, Deal BJ, Johnsrude CL. Fontan conversion to cavopulmonary connection and arrhythmia circuit cryoblation. J Thorac Cardiovasc Surg. 1998;115:547–56. (Reproduced with permission from Elsevier).