Cyanosis in a 9-month-old child after repair of total anomalous pulmonary venous return
A 9-month-old girl presented with cyanosis after correction of total anomalous pulmonary venous return (TAPVR) to the coronary sinus in the neonatal period. During corrective surgery, the right superior vena cava (RSVC) was damaged and re-anastomosed to the right atrium. Echocardiography showed increased flow velocity in the pulmonary venous confluence. Therefore, pulmonary venous obstruction was suspected. However, subsequent cardiac MRI revealed a stenosed RSVC as well as a dilated left superior vena cava (LSVC) draining from the left innominate vein into the pulmonary venous confluence. The re-recruited LSVC drained deoxygenated blood into the systemic circulation, causing cyanosis. After uncomplicated placement of a stent in the narrowed RSVC and occlusion of the LSVC, transcutaneous saturations normalised immediately.
KeywordsCongenital heart disease Magnetic resonance imaging Anomalous pulmonary venous return Paediatric cardiac surgery
Three-dimensional keyhole imaging of the arterial and venous systems. After injection of gadolinium diethylenetriamine penta-acetic acid (Gd-DTPA), the venous phase is imaged with a 3D keyhole technique using maximum intensity projection (MIP) in the anterior-posterior direction (acquisition time, 1.9 s for each 3D dataset; resolution, 2 [overcontiguous slices] × 1 × 1 mm). The venous phase shows the drainage through the reopened left superior vena cava to the left atrium (AVI 8625 kb)
Three-dimensional keyhole imaging of the arterial and venous systems. Same as Movie 1 but the whole 3D dataset is spinning 360 degrees after injection of Gd-DTPA. Using MIP, the venous phase shows the drainage through the reopened LSVC to the left atrium (AVI 32328 kb)