Anomalous pulmonary venous connection: An underestimated entity

Abstract

Anomalous pulmonary venous connection is an uncommon congenital anomaly in which all (total form) or some (partial form) pulmonary veins drain into a systemic vein or into the right atrium rather than into the left atrium. The authors present one case of total anomalous pulmonary venous connection and two cases of partial anomalous pulmonary venous connection, one of supracardiac drainage into the brachiocephalic vein, and the other of infracardiac anomalous venous drainage (scimitar syndrome). Through the presentation of these cases, this article aims to review the main pulmonary venous developmental defects, highlighting the role of imaging techniques in the assessment of these anomalies.

Isolated partial right lower lobe pulmonary venous drainage into the right atrium as a cause of pulmonary hypertension in a middle‐aged woman

“…Cardiac MRI is highly accurate at identifying multiple defects and defining structural abnormalities without radiation. Surgical correction of PAPVC should be considered if the ratio of pulmonary to systemic blood flow is greater than 1.5 or if there is evidence of RV volume overload, regardless of the size of the shunt . Surgical repair includes separating pulmonary venous circulation from the systemic circulation, with anastomosis of the anomalous pulmonary vein or veins to the LA that recreates the normal anatomy.…”

Levoatriocardinal Vein Associated with Pulmonary Stenosis and Aortic Hypoplasia in Young Adult

“…5 Based on the above, the development of pulmonary drainage anomalies begins early during embryogenesis and may occur when any of these stages does not progress adequately. 6 The types of abnormal drainage of pulmonary veins can be divided into four: right anomalous pulmonary venous drainage (APVD), left APVD, scimitar syndrome and levoatriocardinal vein. 5 Anomalous drainage can occur through the superior vena cava, right atrium, inferior vena cava, azygous veins, innominate vein, coronary sinus and hemiazygos veins.…”

Late diagnosis of total anomalous connection of pulmonary veins during the puerperium

“…The total anomalous pulmonary venous connection (TAPVC) is a rare congenital anomaly, which corresponds to approximately 2% of all congenital heart defects [1,2]. The majority of patients present in the neonatal period with decompensated heart failure due to severe pulmonary hypertension or obstruction of pulmonary venous return [3,4]. The natural history of the disease shows that 20% survive until the first year of life without treatment [5].…”