Total anomalous pulmonary venous connection to the portal vein

Abstract

Anomalous pulmonary venous return represents a rare congenital anomaly with wide anatomic and physiologic variability. We report a case of the newborn with a rare form of total infracardiac anomalous pulmonary venous connection (TAPVC). The pulmonary veins draining both lungs formed two vertical veins, which joined to a common pulmonary trunk below the diaphragm. This venous channel connected to the portal vein through the esophageal hiatus. The diagnosis was suggested by color Doppler sonography and confirmed by intravenous digital subtraction angiography, which allowed definition of the anatomy.

Total Anomalous Pulmonary Venous Connection Draining into the Portal Vein in a 2-Month-Old Infant: Multidetector Computed Tomography Angiography Diagnosis

“…5 Total anomalous pulmonary venous connection is generally classified into four types according to the site of connection. 1,3 These four types are also categorised as obstructed or non-obstructed. When it is obstructed, immediate surgery may be required.…”

“…In the evaluation of TAPVC, imaging methods have been used. [1][2][3][4][5] Recent advances Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiac anomaly in which the pulmonary veins fail to connect with the left atrium and connect directly to the right atrium or to one of the systemic veins. Early diagnosis and surgical correction is highly important for survival.…”

Total Anomalous Pulmonary Venous Connection in a Foal

“…The pulmonary veins fail to connect to the left atrium and instead they drain oxygenated blood to systemic veins or directly into the right atrium resulting in a left‐to‐right shunt. An interatrial communication is part of this malformation as all systemic and pulmonary venous blood returns to the right atrium 1,2 . Cyanosis results from a low‐arterial P O 2 as a result of mixing of oxygenated and unoxygenated blood.…”

“…An interatrial communication is part of this malformation as all systemic and pulmonary venous blood returns to the right atrium. 1,2 Cyanosis results from a low-arterial PO 2 as a result of mixing of oxygenated and unoxygenated blood. About 30% of people affected with total anomalous pulmonary venous connection have other major cardiac malformation such as common atrium, single ventricle, or persistent truncus arteriosus.…”

“…The clinical signs of human patients affected with total anomalous pulmonary venous connection depend on the size of the interatrial communication and on the degree of pulmonary vascular resistance, which is often caused by pulmonary venous obstruction. 1,2,9 If the interatrial communication is small, systemic blood flow is markedly limited. In these patients, the right atrial and systemic venous pressures are elevated and hepatic enlargement and peripheral edema develop.…”