ABSTRACT
Partial anomalous pulmonary venous connection (PAPVC) describes abnormal connection of at least one, but not all, pulmonary veins to the right atrium or superior vena cava. It occurs in 0.3-0.6% of all congenital heart diseases. There is a wide anatomic spectrum of PAPVC. The most frequent anomaly is a connection of the right upper and middle lobe pulmonary veins to the right atrium or to the superior vena cava. Abnormal connection of the left-sided pulmonary veins from the left lung may enter, via the brachiocephalic vein, into the superior caval vein or to the coronary sinus. In terms of hemodynamics, a partially anomalous pulmonary venous connection causes a left-to-right shunt and right ventricular volume overload. The degree of left-to-right shunt and the presence of symptoms direct the management. Surgical redirection of the pulmonary veins to the left atrium is the method of choice in the presence of a hemodynamically relevant shunt and/or symptoms.
