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Total anomalous pulmonary venous connection becomes a totallydifferent subset when associated with complex congenital anomalies. The combination of two separate life-threateningcongenital heart defects complicates the management of these patients. Sixpatients with total anomalous pulmonary venous connection associated with complexcongenital heart disease were studied. There were 2 girls and 4 boys. Three ofthem were less than 5 kg in weight, and the other 3 were more than 5 kg inweight. Four patients had severe pulmonary arterial hypertension and 2 patientshad pulmonary stenosis. Three patients had supracardiac type with a rightvertical vein, one had drainage to the right atrium superior vena cavajunction, one patient had supra cardiac type but split flow to both thesuperior vena cava and one patient had cardiac type. Three patients had doubleoutlet right ventricle. Three patients had atrioventricular canaldefect and 2 patients had preoperative pulmonary vein obstruction. All patients underwent rerouting of pulmonaryveins. Concomitant procedures included intraventricular tunnel repair of ventricularseptal defect and infundibular resection in double outlet right ventricle.Atrioventricular canal repair was done for Rastelli type A atrioventricularcanal. Superior vena caval plasty, atrioventricular canal repair and pulmonaryartery banding were done in unbalanced atrioventricular septal defect and large double outlet right ventricle. Intracardiac repair through transatrial approachwas done for tetralogy of Fallot. Right ventricle-pulmonary artery conduit was donefor truncus arteriosus. Single ventricle repair was done for correctedtransposition of great arteries. There were 2 hospital deaths.

KEYWORDS

Total Anomalous; AV Canal; Complex; Congenital; Pulmonary Vein

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B. Karthekeyan, P. Thangavelu, J. Rethinasamy, M. Vakamudi, S. Muthukumar, R. Kodali, K. Sambandham and S. Nandipati -Complex TAPVC-Experience with Six Patients,- Surgical Science, Vol. 4 No. 11, 2013, pp. 509-515. doi: 10.4236-ss.2013.411099.





Author: Baskar Ranjith Karthekeyan, Periyasamy Thangavelu, Jebaraj Rethinasamy, Mahesh Vakamudi, Siva Muthukumar, Rajeshkumar Kodali, Kam

Source: http://www.scirp.org/



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