Pulmonary vein stenosis following repair of total anomalous pulmonary venous connection.
ABSTRACT Pulmonary vein stenosis may occur following the repair of total anomalous pulmonary venous connection and carries a poor prognosis. Conventional surgical therapies have been complicated by a high rate of re-stenosis. Sutureless pericardial marsupialization has been introduced as a technique to reduce the rate of re-stenosis. This report describes a retrospective review of a group of patients undergoing repair of acquired pulmonary vein stenosis. Presence of single-ventricle anatomy was found to be the primary preoperative risk factor for a poor outcome. The use of sutureless pericardial marsupialization was found to be associated with a significant improvement in disease-free survival.
- Korean Journal of Pediatrics 01/2009; 52(2).
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ABSTRACT: Although improved surgical techniques have led to significantly better outcomes of surgery for total anomalous pulmonary venous connection, the risk of progressive pulmonary venous obstruction continues to be a clinical problem. Both obstructed total anomalous pulmonary venous connection and post-repair pulmonary venous obstruction are associated with a significant risk of recurrent obstruction or death, requiring reoperation for stenosis. In general, side to side anastomosis of the pulmonary venous confluence to the functional left atrium has been performed for supracardiac and infracardiac total anomalous pulmonary venous connection. Repair of total anomalous pulmonary venous connection to the coronary sinus invariably involved unroofing the coronary sinus, followed by pericardial patch closure of the atrial septal defect. Recently, sutureless technique has been adopted as the primary operation for the subgroups of patients that are thought to be at high risk for post-repair pulmonary venous obstruction, such as those with total anomalous pulmonary venous connection associated with right isomerism, infracardiac total anomalous pulmonary venous connection with small individual pulmonary veins, or mixed-type total anomalous pulmonary venous connection. Because the sutureless technique does not require direct anastomosis to the confluence, aggressive resection of the obstructed pulmonary venous tissue can be achieved, and surgically induced distortion of the suture line can be avoided, which may help to prevent subsequent pulmonary venous obstruction. Conventional management strategies for recurrent pulmonary venous obstruction have typically been associated with poor outcomes. Recent reports have supported the use of the sutureless technique to treat post-repair pulmonary venous obstruction.General Thoracic and Cardiovascular Surgery 10/2012;
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ABSTRACT: We present the case of a newborn with severe pulmonary hypertension, diagnosed with infradiaphragmatic type of total anomalous pulmonary venous connection (TAPVC). The onset was in the first 10 days of life. Diagnosis was made by echocardiography and AngioCT. The pulmonary venous collector was surgically implanted into the left atrium in Germany, but the next month after surgery he developed cardiopulmonary insufficiency and died several days later. We would like to emphasize the importance of prostaglandin E1 administration in this particular case of infradiaphragmatic type of TAPVC and its usefulness in patient's stabilization until surgery. The prognosis in TAPVC, infradiaphragmatic type, is poor and is related mainly to the existence of pulmonary venous obstruction.Mædica. 06/2012; 7(2):167-72.