Total anomalous pulmonary venous return is a congenital (meaning it’s present from birth) heart disease that affects the pulmonary veins.

Arteries carry blood away from the heart; veins carry it toward the heart. The pulmonary veins are very important: they carry the “red” blood that has picked up oxygen in the lungs back to the heart to be pumped out to the body. There are typically four pulmonary veins, two from each lung. Normally, they all connect directly into the heart’s left atrium (left upper chamber).

In a child born with total anomalous — which means abnormal — pulmonary venous return (TAPVR), the pulmonary veins connect to other veins and ultimately drain their blood into the right atrium. Normally, the right atrium only receives “blue” blood coming back from the body without oxygen, which then passes into the right ventricle and is pumped to the lungs. In TAPVR, the right atrium receives both “blue” blood from the body and “red” blood from the lungs.

As a result, the right side of the heart is overworked from pumping the extra blood it receives. Frequently, there is blockage to the veins that receive blood from the pulmonary veins, which causes a backup of blood in the lungs. This disrupts the normal flow of blood between the lungs and the body. Additionally, the blood leaving the heart from the body has a lower level of oxygen.

Children with TAPVR also have other heart defects. They have a hole in the wall separating the two upper chambers of the heart (atrial septal defect) and may have a patent ductus arteriosus, an extra blood vessel between the pulmonary arteries and the aorta. These heart defects can actually help the child with TAPVR survive by allowing more blood to get from the right side of the heart to the left side and out to the body. In fact, children without at least an atrial septal defect will not survive.

Our pediatric cardiologists will explain your child’s heart anatomy in detail. Generally, there are four types of TAPVR.” Read More