Pulmonary Venous Anomalies

Anomalous connection may involve all or some of the pulmonary veins. The former is known as totally anomalous pulmonary venous connection (TAPVC), while the latter is known as partially anomalous pulmonary venous connection (PAPVC). In this regard, the venous connections can be anatomically appropriate, yet the drainage can still be anomalous, as in the setting of fenestration of the coronary sinus, or the so-called “levoatrial cardinal vein.” The presentation of the anomalous connections, as would be expected, varies significantly between the two lesions. In PAPVC, the clinical presentation is amplified by the number of involved pulmonary veins. An additional modifier of clinical presentation is governed by the potential association of pulmonary venous stenosis, which if present may lead to significant pulmonary disease. Finally, as is the case in most congenital heart diseases, anomalies of pulmonary venous drainage of connection may be isolated or found as part of a more complex disease involving anomalies of other components of the cardiovascular system or the extracardiac organs. In this chapter, we review both types of anomalous pulmonary venous connections and their subtypes.