Surgical Treatment for Asplenia Syndrome / 日本心臓血管外科学会雑誌

ABSTRACT

Recently, modified Fontan operation is being used for asplenia syndrome. We reviewed 24 patients with asplenia syndrome who underwent surgical intervention. Eleven (45.8%) of them had total anomalous pulmonary venous connection (TAPVC) and 6 of these were accompanied by pulmonary venous obstruction (PVO). Surgical results were poor in the PVO group. In 4 cases with open heart palliation, that is atrium-common pulmonary venous chamber anastomosis, there were 2 operative deaths, 1 late death, and 1 survival. In 18 cases without PVO, statistical analysis (Fisher's exact probability) showed that pulmonary atresia (PA) was a definite risk factor for Fontan candidates (<i>p</i><0.05). In 9 cases with pulmonary stenosis (PS group), there were 5 candidates for the Fontan type operation. In the other 9 cases with PA (PA group) there were no candidates for the Fontan type operation. Only the size and the morphology of the pulmonary artery were significant factors (<i>p</i><0.05) for candidates of the Fontan type operation among the risk factors such as size and morphology of the pulmonary artery, pulmonary vascular resistance and pressure, atrioventricular valve regurgitation, and single ventricular function. Finally, in these 24 cases, there were only 5 candidates (20.8%) for a Fontan type operation. In conclusion, in order to increase candidates for Fontan precedures, it is important to maintain an adequate pulmonary blood flow. Earlier PDA division and pulmonary arteries plasty are the most importantin PA group. In both groups pulsatile bidirectional cavopulmonary shunts may be useful to increase effective pulmonary blood flow without ventricular volume overload, which leads to atrioventricular valve regurgitation.