Total Anomalous Pulmonary Venous Return

Document Type

Book Chapter

Publication Date



DOI: 10.1016/B978-1-4557-0760-7.00048-6


Total anomalous pulmonary venous connection (TAPVR) is a rare lesion with the pulmonary veins commonly connecting to a venous confluence that ultimately drains to the desaturated atrial chamber. Long-term medical management results in poor survival, making surgical intervention necessary. Correction during the newborn period is associated with low mortality and excellent mid-term outcomes. Diagnostic advances now provide better visualization of these defects than ever before, allowing accurate and detailed preoperative definition of the anomalous anatomy and physiology. Infants who present with obstruction in the immediate neonatal period require emergent operation and often face a difficult postoperative course, but long-term survival has significantly improved. Surgical repair for infants with less severe cases can be performed electively, but there is evidence to suggest that early repair may result in improved postoperative outcomes. Recurrent pulmonary venous stenosis is a vexing common postoperative morbidity, occurring in up to 25% of TAPVR patients. There are multiple surgical techniques in use to decrease these morbidities, though controversy regarding the optimal method persists. Overall, surgical outcomes for TAPVR have greatly improved, and patients with this condition appear to enjoy excellent long-term functional status today.

Journal Title

Critical Heart Disease in Infants and Children (Third Edition)

First Page


Last Page



total anomalous pulmonary venous connection; congenital heart defect; pulmonary venous stenosis; pediatric; critical care

Library Record