OBJECTIVE--To assess the effect of the condition of the right heart after the Fontan operation on portal venous flow, and to determine whether the characteristics of portal venous flow were different when there was an atriopulmonary connection with atrial septal closure rather than an atriopulmonary or total cavopulmonary connection with intra-atrial routing. PATIENTS AND METHODS--After the Fontan operation six patients with an atriopulmonary connection (group 1), three patients with an atriosubpulmonary connection (group 2), four patients with intra-atrial routing and an atriopulmonary connection (group 3), and five patients with a total cavopulmonary connection (group 4) were studied by pulsed Doppler echocardiography. The flow signals were recorded for the pulmonary artery, hepatic vein, and intrahepatic portal vein in each patient. Postoperative cardiac catheterisation was performed in 16 of the 18 patients. The Doppler findings were compared with those of 14 controls. RESULTS--The portal flow was pulsatile in 13 patients and constant in five patients. Reversed flow was shown at or just after the QRS wave after hepatic venous regurgitation in two group 1 patients. The flow signal was interrupted in two group 1 patients and two group 2 patients. Decrease in velocity of flow was recorded in two group 1 patients, one group 2 patients, three group 3 patients, and one group 4 patient. Portal flow was constant in one group 3 patient and four group 4 patients. The pulsatility ratio ranged from -0.46 to 0.49 (mean (SD), 0.03 (0.32)) in the patients from group 1 and 2, from 0.41 to 0.76 (0.54 (0.15)) in group 3, and from 0.70 to 0.80 (0.75 (0.04)) in group 4. The ratio in the controls ranged from 0.29 to 0.83 (0.61 (0.13)). The ratio was significantly lower in groups 1 and 2 than in group 3 (p < 0.01), group 4 (p < 0.01), or the controls (p < 0.005). There was no significant difference in the ratio between group 3 and group 4 and no correlation between the pulsatility ratio and the cardiac index (r = 0.26), mean right atrial pressure (r = 0.25), or pulmonary vascular resistance (r = 0.17). CONCLUSION--The larger hepatic venous regurgitation caused by atrial contraction in patients with an atriopulmonary connection correlated with the higher portal pulsatility and a total cavopulmonary connection reduced portal pulsatility.
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