Objective: We investigated the subdiaphragmatic venous physiology in patien
ts subjected to the Fontan operation to understand some of the early and la
te problems of this circulation.
Methods: Flows were evaluated by Doppler ultrasonography in the subhepatic
inferior vena cava, hepatic vein, and portal vein during respiratory monito
ring and with a tilt table. Twenty control subjects (group A) and 56 patien
ts who had the Fontan operation, 27 in functional class I(group B) and 29 i
n class III or IV (group C), were studied. Inspiratory/expiratory flow rati
o was calculated to reflect respiratory effects, and upright/supine flow ra
tio was calculated to assess gravity effects. Inferior vena caval, hepatic
venous, and wedged hepatic venous pressures were measured during catheteriz
ation in 21 control subjects and 25 Fontan patients. The difference between
wedged and hepatic venous pressures represents the transhepatic venous pre
ssure gradient.
Results: Fontan hepatic venous flow depended more on inspiration than contr
ol, but without difference between groups B and C (inspiratory/expiratory f
low ratios: 1.7, 2.9, and 2.9, respectively; P < .02). Normal portal venous
flow was higher in expiration; this effect was lost in group B and reverse
d in group C (inspiratory/expiratory flow ratios: 0.8, 1.0, and 1.3; P < .0
005). Gravity reduced portal venous flow in groups A and B, but progression
to functional class III or IV (group C) exacerbated this effect (upright/s
upine flow ratios: 0.8, 0.7, and 0.5; P < .01). Inferior vena caval, hepati
c venous, and wedged hepatic venous pressures tin millimeters of mercury) i
n the Fontan groups were all elevated compared with the control group (infe
rior vena cava, 14.4 <plus/minus> 4.4 vs 5.9 +/- 2.3; hepatic vein, 14.7 +/
- 4.5 vs 5.9 +/- 1.9; wedged hepatic vein, 14.7 +/- 4.0 vs 8.3 +/- 2.6; P <
.0001). However, trans hepatic venous pressure gradient in the Fontan grou
p was lower than in the control group (0.5 <plus/minus> 0.5 vs 2.3 +/- 2.0;
P < .001). Univariate analysis of inferior vena caval pressure and transhe
patic venous pressure gradient showed significant inverse correlation (r =
0.6, P < .002).
Conclusions: In patients who are in functionally poorer condition after the
Fontan operation, portal venous flow loses normal expiratory augmentation
and adverse gravity influence is enhanced. These suboptimal flow dynamics,
coupled with higher splanchnic venous pressures and lower transhepatic veno
us pressure gradients, suggest that hepatic sinusoids are congested, acting
as "open tubes." Transhepatic gradient loss is incrementally worse with hi
gher caval pressures. These observations may be responsible for late gastro
intestinal problems in patients who have had the Fontan operation.