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Review
. 2005 Jun;91(6):839-46.
doi: 10.1136/hrt.2004.051789.

The Fontan circulation

Marc Gewillig  1
Affiliations
Review

The Fontan circulation

Marc Gewillig. Heart. 2005 Jun.
No abstract available

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Figures

Figure 1
Figure 1
(A) The normal cardiovascular circulation. The pulmonary circulation (P) is connected in series with the systemic circulation (S). The right ventricle maintains the right atrial pressure lower than the left atrial pressure, and provides enough energy to the blood to pass the pulmonary resistance. (B) The patient with a univentricular heart. The systemic and pulmonary circuits are connected in parallel, with a considerable volume overload to the single ventricle (V). The width of the line reflects the degree of volume load. There is complete admixture of systemic and pulmonary venous blood, causing arterial oxygen desaturation. (C) The Fontan circulation. The systemic and pulmonary circulations are connected in series. The right atrium (RA) or systemic veins are connected to the pulmonary artery (PA). The volume overload to the single ventricle is now less than expected for body surface area. In the absence of fenestration, there is no more admixture of systemic and pulmonary venous blood, but the systemic venous pressure is notably elevated. Ao; aorta; LA, left atrium; LV, left ventricle, RV, right ventricle.
Figure 2
Figure 2
Schematic representation of cardiac malformations that are suitable for Fontan repair. Top left: tricuspid atresia. Top right: double inlet left ventricle. Bottom left: hypoplastic left heart syndrome. Bottom right: unbalanced atrioventricular septum defect.
Figure 3
Figure 3
Angiogram of total cavopulmonary connection. (A) Injection in inferior caval vein which is connected with a 22 mm Goretex graft to the pulmonary artery; note mild preferential flow to left pulmonary artery. (B) Injection in right superior caval vein which is connected to the right pulmonary artery; note mild preferential flow to right pulmonary artery.
See this image and copyright information in PMC

References

    1. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax 1971;26:240–8. ▸ The original description of the first “Fontan” operation: a historical landmark, however with a circuit which nowadays would be considered obsolete. - PMC - PubMed
    1. Gewillig M, Kalis N. Pathophysiologic aspects after cavo-pulmonary anastomosis. Thorac Cardiovasc Surg 2000;48:336–41. ▸ Extensive review of the effects of different pre-Fontan treatment strategies on ventricular size and function. - PubMed
    1. Choussat A, Fontan F, Besse F, et al. Selection criteria for Fontan’s procedure. In: Anderson R, Shinebourne E, eds. Paediatric cardiology. Edinburgh: Churchill Livingstone, 1978:559–66.
    1. De Leval M, Kilner P, Gewillig M, et al. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg 1988;96:682–95. ▸ Elegant discussion on advantages of cavopulmonary connections versus atriopulmonary connection. - PubMed
    1. Bridges ND, Lock JE, Castaneda AR. Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk. Circulation 1990;82:1681–9. - PubMed