Background: Both pulmonary and systemic circulation must be maintained by a
single pump in Fontan circulation. This unique property of Fontan circulat
ion may be related to decreased exercise tolerance or increased instantaneo
us postoperative mortality rate, often observed in patients with this circu
lation. To better understand Fontan physiology, the present study theoretic
ally investigated cardiac performance of Fontan circulation by using ventri
cular-vascular coupling framework analysis.
Methods: End-systolic volume elastance (Ees), as a chamber contractile prop
erty, and effective arterial elastance (Ea), a lumped measure of ventricula
r afterload, were estimated both in normal left ventricular systemic circul
ation and in Fontan circulation.
Results: End-systolic volume elastance was decreased and Ea was increased i
n Fontan circulation. Both ventricular external stroke work (SW) and mechan
ical efficiency (EFF) under Fontan circulation were lower compared with tho
se under normal circulation. Furthermore, the Ees-Ea relationship in Fontan
circulation predicted limited cardiac reserve in terms of SW and EFF. Such
cardiac performance in Fontan circulation stemmed from increased impedance
due to the additional connection of the pulmonary vascular bed to the syst
emic vasculature and from the lack of a compensatory increase in contractil
ity for increased afterload.
Conclusions: Thus, it was inferred that Fontan circulation had intrinsic di
sadvantages and this may explain, in part, abnormal functional status and d
ecline in survival following this procedure.