Bilateral Vertical Veins From a Common Confluence in Supracardiac Total Anomalous Pulmonary Venous Connection
We present a case of unusual pulmonary venous anatomy, consisting of 2 separate vertical veins, in a newborn with supracardiac total anomalous pulmonary venous connection (TAPVC). A 3.0-kg neonate boy presented on day 6 of life with pulmonary edema. He was diagnosed by echocardiography as having obstructed supracardiac TAPVC, a patent arterial duct, and a secundum-type atrial septal defect. All left- and right-sided pulmonary veins drained into a confluence behind the left atrium. This confluence then connected via 2 separate vertical veins to the left innominate vein (IV) superiorly (Figure 1 and online-only Data Supplement Movie). The left-sided vertical vein coursed from inferior to superior to drain close to the left internal jugular vein and was stenotic above the level of the left pulmonary artery (Figure 2A and online-only Data Supplement Movie). The right-sided vertical vein ran more anteriorly in an oblique course from left to right to drain into the left IV at the superior vena cava junction. This vein was obstructed at its entrance into the left IV (Figures 1 and 2⇓B).
The defect was repaired by surgical anastomosis of the pulmonary venous confluence to the left atrium and ligation of both vertical veins. The arterial duct was ligated and the atrial septal defect closed. The echocardiographic findings were confirmed at surgery.
TAPVC is categorized as supracardiac, cardiac, intracardiac, or mixed forms.1 Supracardiac TAPVC is the result of failed development or atresia of the common pulmonary vein, which connects the embryonic lung buds with the left atrium.1 Collateral channels for pulmonary venous drainage develop via the fetal cardinal venous system. Usually, only 1 of these channels persists to become the vertical vein that connects the pulmonary venous confluence to the superior vena cava or the IV. Considerable variability exists in the pulmonary to systemic venous connection in all forms of TAPVC2; in the heterotaxy syndromes, separate vertical veins draining blood from the left and the right lung are well recognized.3 However, to the best of our knowledge, this is the first report of a right and left vertical vein emerging from a single confluence that receives the total pulmonary venous return.
The online-only Data supplement, which consists of a movie, is available with this article at http://circ.ahajournals.org/cgi/content/full/118/6/e103/DC1.
Geva T, van Praagh S. Anomalies of the pulmonary veins. In: Allen H, Gutgesell H, Clark E, Driscoll D, eds. Moss and Adams’ Heart Disease in Infants, Children, and Adolescents. 6th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2001: 736–772.
Cheung YF, Cheng VY, Chau AK, Chiu CS, Yung TC, Leung MP. Outcome of infants with right atrial isomerism: is prognosis better with normal pulmonary venous drainage? Heart. 2002; 87: 146–152.