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• W2005806898 abstract "A 5-year-old child was referred for persistent cyanosis (oxygen saturation of 88%) after an episode of pneumonia. The anteroposterior chest roentgenogram showed a dilatation of the right upper mediastinum (*) (Fig 1A). The transthoracic echocardiogram, subcostal projection, revealed an obstructive supracardiac total anomalous pulmonary venous connection (TAPVC); all pulmonary veins connected with the innominate vein through a vertical vein. However, the innominate vein was significantly narrowed distally, close to the innominate/right superior vena cava (SVC) junction, and the SVC was severely dilated (poststenotic turbulence at the innominate/SVC junction; Fig 1B).Cardiac catheterization confirmed the diagnosis (the angiographic gradient at the level of the obstruction was 20 mm Hg; the arrow indicates the obstruction in Fig 1C; Ao = ascending aorta, I = innominate vein; RPV = right pulmonary vein, V = vertical vein). The main pulmonary to systemic mean arterial blood pressure ratio was 0.7, and the pulmonary vascular resistance was normal. At operation (severely dilated SVC; Fig 2), several enlarged lymph nodes were found extrinsically compressing the distal innominate vein at the junction with the SVC. Operative repair included (1) anastomosis of the horizontal confluence of the pulmonary veins to the back wall of the left atrium, (2) reconstruction of the interatrial septum with a synthetic patch that was also used to augment the size of the left atrium, (3) ligation of the vertical vein, and (4) resection of the enlarged lymph nodes that extrinsically compressed the innominate vein. The patient was weaned without difficulties from cardiopulmonary bypass, in sinus rhythm, with a pulmonary to systemic mean arterial blood pressure ratio of 0.3. The child had an uncomplicated postoperative course and was discharged on the fifth postoperative day.The postoperative echocardiogram showed unobstructed flow from all 4 pulmonary veins into the left atrium and no residual gradient between the jugular vein and the SVC. The presence of obstruction at the level of the innominate vein in supracardiac TAPVC with drainage of the pulmonary veins through a vertical vein is extremely rare. The obstruction in this case was caused by enlarged lymph nodes, most probably secondary to the child’s recent pneumonia. This mechanism could also explain the normal pulmonary vascular resistance in this child before and after the operation.We believe this to be a very rare case of obstructive supracardiac TAPVC, with extrinsic compression of the innominate vein and a poststenotic giant SVC. A 5-year-old child was referred for persistent cyanosis (oxygen saturation of 88%) after an episode of pneumonia. The anteroposterior chest roentgenogram showed a dilatation of the right upper mediastinum (*) (Fig 1A). The transthoracic echocardiogram, subcostal projection, revealed an obstructive supracardiac total anomalous pulmonary venous connection (TAPVC); all pulmonary veins connected with the innominate vein through a vertical vein. However, the innominate vein was significantly narrowed distally, close to the innominate/right superior vena cava (SVC) junction, and the SVC was severely dilated (poststenotic turbulence at the innominate/SVC junction; Fig 1B). Cardiac catheterization confirmed the diagnosis (the angiographic gradient at the level of the obstruction was 20 mm Hg; the arrow indicates the obstruction in Fig 1C; Ao = ascending aorta, I = innominate vein; RPV = right pulmonary vein, V = vertical vein). The main pulmonary to systemic mean arterial blood pressure ratio was 0.7, and the pulmonary vascular resistance was normal. At operation (severely dilated SVC; Fig 2), several enlarged lymph nodes were found extrinsically compressing the distal innominate vein at the junction with the SVC. Operative repair included (1) anastomosis of the horizontal confluence of the pulmonary veins to the back wall of the left atrium, (2) reconstruction of the interatrial septum with a synthetic patch that was also used to augment the size of the left atrium, (3) ligation of the vertical vein, and (4) resection of the enlarged lymph nodes that extrinsically compressed the innominate vein. The patient was weaned without difficulties from cardiopulmonary bypass, in sinus rhythm, with a pulmonary to systemic mean arterial blood pressure ratio of 0.3. The child had an uncomplicated postoperative course and was discharged on the fifth postoperative day. The postoperative echocardiogram showed unobstructed flow from all 4 pulmonary veins into the left atrium and no residual gradient between the jugular vein and the SVC. The presence of obstruction at the level of the innominate vein in supracardiac TAPVC with drainage of the pulmonary veins through a vertical vein is extremely rare. The obstruction in this case was caused by enlarged lymph nodes, most probably secondary to the child’s recent pneumonia. This mechanism could also explain the normal pulmonary vascular resistance in this child before and after the operation. We believe this to be a very rare case of obstructive supracardiac TAPVC, with extrinsic compression of the innominate vein and a poststenotic giant SVC." @default.
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• W2005806898 date "2006-03-01" @default.
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• W2005806898 title "Rare Case of Obstructive Supracardiac Total Anomalous Pulmonary Venous Connection in Association With a Giant Superior Vena Cava" @default.
• W2005806898 doi "https://doi.org/10.1016/j.athoracsur.2004.04.067" @default.
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