Abstract
Total anomalous pulmonary venous return (TAPVR) is a cardiac malformation defined by a complete absence of communication between the pulmonary veins and the left atrium. All oxygenated pulmonary venous return flows directly or indirectly into the right atrium. Survival is only possible via a right-to-left atrial shunt. Non-blocked forms present as high-flow shunts with moderate cyanosis. Symptoms in blocked forms are dominated by obstruction of venous return, and life-threatening distress with intense cyanosis and severe pulmonary hypertension sets in from the very first days of life. Treatment consists in connecting the pulmonary venous receptacle to the left atrium, tying off the collector and closing the inter-atrial septum. The risks of surgery remain high in severe forms, and failures usually occur in the first year of life. In the case of good surgical correction, the long-term prognosis is excellent.
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