Pulmonary hepatic flow distribution in total cavopulmonary connections: Extracardiac versus intracardiac

ObjectivePulmonary arteriovenous malformations can occur after the Fontan procedure and are believed to be associated with disproportionate pulmonary distribution of hepatic venous effluent. We studied the effect of total cavopulmonary connection geometry and the effect of increased cardiac output on distribution of inferior vena caval return to the lungs.MethodsTen patients undergoing the Fontan procedure, 5 with extracardiac and 5 with intracardiac configurations of the total cavopulmonary connection, previously analyzed for power loss were processed for calculating the distribution of inferior vena caval return to the lungs (second-order accuracy). One idealized total cavopulmonary connection was similarly analyzed under parametric variation of inferior vena caval offset and cardiac output flow split.ResultsStreaming of the inferior vena caval return in the idealized total cavopulmonary connection model was dependent on both inferior vena caval offset magnitude and cardiac output flow-split ratio. For patient-specific total cavopulmonary connections, preferential streaming of the inferior vena caval return was directly proportional to the cardiac output flow-split ratio in the intracardiac total cavopulmonary connections (P < .0001). Preferential streaming in extracardiac total cavopulmonary connections correlated to the inferior vena caval offset (P < .05) and did not correlate to cardiac output flow split. Enhanced mixing in intracardiac total cavopulmonary connections is speculated to explain the contrasting results. Exercising tends to reduce streaming toward the left pulmonary artery in intracardiac total cavopulmonary connections, whereas for extracardiac total cavopulmonary connections, exercising tends to equalize the streaming.ConclusionsExtracardiac and intracardiac total cavopulmonary connections have inherently different streaming characteristics because of contrasting mixing characteristics caused by their geometric differences. Pulmonary artery diameters and inferior vena caval offsets might together determine hepatic flow streaming.