Abstract
Total anomalous pulmonary venous connection becomes a totally different subset when associated with complex congenital anomalies. The combination of two separate life-threatening congenital heart defects complicates the management of these patients. Six patients with total anomalous pulmonary venous connection associated with complex congenital heart disease were studied. There were 2 girls and 4 boys. Three of them were less than 5 kg in weight, and the other 3 were more than 5 kg in weight. Four patients had severe pulmonary arterial hypertension and 2 patients had pulmonary stenosis. Three patients had supracardiac type with a right vertical vein, one had drainage to the right atrium superior vena cava junction, one patient had supra cardiac type but split flow to both the superior vena cava and one patient had cardiac type. Three patients had double outlet right ventricle. Three patients had atrioventricular canal defect and 2 patients had preoperative pulmonary vein obstruction. All patients underwent rerouting of pulmonary veins. Concomitant procedures included intraventricular tunnel repair of ventricular septal defect and infundibular resection in double outlet right ventricle. Atrioventricular canal repair was done for Rastelli type A atrioventricular canal. Superior vena caval plasty, atrioventricular canal repair and pulmonary artery banding were done in unbalanced atrioventricular septal defect and large double outlet right ventricle. Intracardiac repair through transatrial approach was done for tetralogy of Fallot. Right ventricle-pulmonary artery conduit was done for truncus arteriosus. Single ventricle repair was done for corrected transposition of great arteries. There were 2 hospital deaths.
Highlights
Total anomalous pulmonary venous connection is characterized by the failed union of the pulmonary veins and incorporation of it by the developing left atrium in combination with a persistent embryologic connection between the pulmonary and systemic venous systems
Six patients with total anomalous pulmonary venous connection associated with complex congenital heart disease were diagnosed in our hospital between 2011 and 2013
Improvement or modification of the primary repair technique is required to avoid postoperative pulmonary vein obstruction, such as using sutureless technique, especially in patients of whom total anomalous pulmonary venous connection is associated with complex congenital cardiac anomalies
Summary
Total anomalous pulmonary venous connection is characterized by the failed union of the pulmonary veins and incorporation of it by the developing left atrium in combination with a persistent embryologic connection between the pulmonary and systemic venous systems. The impact of the pathology depends on the degree to which the pulmonary venous drainage is obstructed and the magnitude of the left-to-right shunt. Accurate assessment of the anatomy and detailed surgical planning become essential when total anomalous pulmonary venous connection is associated with other complex congenital anomalies. This result in a combination of two separate lifethreatening heart defects needs appropriate management. Most often the timing of surgery is dictated by the presence or development of pulmonary venous obstruction
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
