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A 4 year old boy with double inlet left ventricle, tricuspid atresia, and malposition of the great arteries had initially undergone banding of the pulmonary artery. At the age of 5 months a bidirectional Glenn anastomosis was placed. Finally, definite single ventricle palliation was achieved by creation of a fenestrated extracardiac total cavopulmonary connection (TCPC). At the same time, the stenotic left pulmonary artery was augmented by means of a patch plasty. Low dose prophylactic anticoagulation was started. Four months later, pulse oximetry readings had dropped to about 5% below his baseline. The chest x ray revealed preferential pulmonary blood flow to the right. While neither the TCPC baffle nor the branch pulmonary arteries were sufficiently seen by echocardiography, magnetic resonance imaging clearly visualised the intra- and extracardiac anatomy. The extracardiac baffle was unobstructed as was the right pulmonary artery. However, the left pulmonary artery showed a severe long segment narrowing (panel). An additional patch was placed to augment the vessel size.
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