Pulmonary Venous Abnormalities Rachel D Vanderlaan, Robert H Anderson, Girish Shirali, Diane E Spicer, Anthony M Hlavacek, Christopher A Caldarone Abstract Pulmonary venous abnormalities comprise a wide spectrum of congenital lesions Total anomalous pulmonary venous connection is the most common and constitutes a spectrum of congenital lesions in which the pulmonary veins remain connected to systemic venous vessels or aberrantly connect to the right atrium Definitive management requires surgical intervention; in patients with obstruction to pulmonary venous flow, urgent operation is required The use of temporizing catheter-based interventions allows for optimization in hemodynamically unstable neonates Other pulmonary venous conditions, such as partial anomalous pulmonary venous connections, require surgical corrections but on an elective basis Overall, survival for anomalous pulmonary venous abnormalities has significantly improved over the past decades through better perioperative management and the evolution of surgical and catheter-based approaches Postrepair and congenital pulmonary venous stenosis persists as major determinants of long-term outcomes Keywords Anomalous pulmonary veins; pulmonary vein stenosis; atresia; sutureless repair; obstructed TAPVC Introduction This chapter considers abnormalities of the pulmonary veins, in particular their anomalous connections to the systemic venous system The variant of partially anomalous pulmonary venous connection associated with the sinus venosus interatrial communication is described in detail The anomalous pulmonary venous connections so commonly associated with isomerism of the atrial appendages are mentioned in this chapter but are discussed more fully in Chapter 26, not least because of the particular problems of nomenclature that arise in the setting of isomerism Although division of the morphologically left atrium was, in the past,1 considered a pulmonary venous anomaly, this lesion, along with division of the morphologically right atrium, is covered in Chapter 30