Asplenia syndrome with total anomalous pulmonary venous connection
Noma, M.; Hiramatsu, Y.; Tokunaga, C.; Imazuru, T.; Matsushita, S.; Shigeta, O.; Sakakibara, Y.; Horigome, H.; Takahashi, M.
Kyobu Geka. Japanese Journal of Thoracic Surgery 58(2): 133-136
2005
ISSN/ISBN: 0021-5252 PMID: 15724476 Document Number: 585558
Three patients of asplenia syndrome with total anomalous pulmonary venous connection (TAPVC) were reported. Case 1 with exceeding pulmonary blood flow, underwent TAPVC repair and pulmonary artery banding as a first palliation before bi-directional Glenn shunt. Case 2 did not require any surgery to control the pulmonary blood flow before the simultaneous procedure of TAPVC repair and bi-directional Glenn shunt. Case 3 with decreased pulmonary blood flow underwent a complicated course with 3 times of Blalock-Taussig shunts and the repair of TAPVC to prepare for bi-directional Glenn shunt. Simultaneous repair of TAPVC with the procedure which aimed to control the pulmonary blood flow at the first palliation surgery will simplify the control of pulmonary blood flow and prepare good condition of the lung for the Fontan operation in the future.