Abstract
The Fontan circulation provides definite palliation for children born with a single anatomical or functional ventricle by diverting systemic venous blood directly to the pulmonary arteries, effectively rendering systemic venous return into portal vessels to the lung. Although this restores pulmonary blood flow and avoids the mixture of oxygenated and deoxygenated blood, it also results in elevated systemic venous pressures and low cardiac output. These are the two hallmarks of any Fontan circulation and the cause of Fontan circulatory failure later in life. We highlight the determinants of systemic venous return, its changed relationship with the pulmonary circulation, how it affects preload, and the changed role of the heart (myocardium, valves, and heart rate). By critically evaluating the components of the Fontan circulation, we hope to give some clues in how to optimize the Fontan circulation and avenues for future research.
Highlights
Francois Fontan and Eugene Baudet’s pioneering work on the surgical treatment of patients with tricuspid atresia eventually led to the development of the Fontan operation [1]
We have shown that the respiratory pump in Fontan patients causes a respiratory-induced variation in stroke volume which is only partly attenuated by the pulmonary circulation [15]
The Fontan portal system redefines the place of the pulmonary circulation as the main factor influencing systemic venous return and cardiac output
Summary
Francois Fontan and Eugene Baudet’s pioneering work on the surgical treatment of patients with tricuspid atresia eventually led to the development of the Fontan operation [1]. The surgeons’ main aim was to restore pulmonary blood flow and to eliminate the mixture of venous and oxygenated blood Whilst this remains crucial to the concept of the Fontan circulation, Drs Fontan and Baudet’s hypothesis that this required some form of an atrial (or ventricular) “pump” was later superseded when de Leval nicely illustrated that the right atrium as a valveless chamber does not contribute to blood flow at the higher venous pressures observed in Fontan patients. While the Fontan operation became the final palliation for thousands of patients with a single anatomical or functional ventricle worldwide, pediatric and adult congenital cardiologists are increasingly confronted with the limitations posed by the circulation created many years ago [3–5]. Since innovation follows understanding based on simple observation, the case of the failing Fontan is not necessarily hopeless
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