Imaging Features of the Total Anomalous Pulmonary Venous Connection to the Portal Vein Shown by Multidetector Computed Tomography Angiography

Abstract

A 2-month-old girl with ventriculoseptal defect and pulmonary hypertension (PHT) presented with respiratory distress and coughing. Peroral cyanosis, tachypnea, dyspnea, growth retardation, systolic murmur, crepitant rales in both lungs, and hepatomegaly were discovered at the physical examination. A chest roentgenogram showed a normal-sized heart and mild venous congestion. Echocardiography exhibited a small perimembranous ventriculoseptal defect. The pulmonary veins were draining into the portal vein through a vertical vein. Angiography showed drainage of the pulmonary veins into a portal vein via a vertical vein. The superior vena cava and right atrium were filling via an innominate vein. Multidetector computed tomography (MDCT) angiography showed that the right and the left pulmonary veins were joined at the anterior side of the proximal descending aorta, forming the vertical vein. The latter connected to the portal confluens, with the splenic vein and superior mesenteric vein below the diaphragm (Figs. 1 and 2). There also was hepatomegaly, but no congenital anomaly of the pulmonary arteries and lungs was observed. Despite conservative therapy, the girl died in 2 weeks because of congestive heart failure and pneumonia. The infradiaphragmatic total anomalous pulmonary venous connection (TAPVC) is almost always obstructed or narrowed when it passes through the diaphragm. However, no obstruction was observed in the reported case. Because no congenital anomaly was found in the lungs or pulmonary arteries and because the pulmonary venous return was not partial, ‘‘scimitar syndrome’’ was easily excluded. The snowman figure seen on the chest radiographs is due to enlargement of the left vertical vein and superior vena cava and characteristic of type 1 TAPVC. In contrast, other types of TAPVC show nonspecific roentgenographic findings. Nonobstructive types include cardiomegaly with right atrial and right ventricular enlargement, an enlarged pulmonary vein, and increased pulmonary flow. Obstructive types demonstrate