In double-outlet right ventricle (DORV) — a heart condition present at birth (congenital) — the main artery that carries blood from the heart to the body (aorta) and the artery that directs blood from the heart to the lungs (pulmonary artery) are partially or completely connected to the lower right heart chamber (right ventricle). Sometimes these blood vessels are also reversed from their normal positions (transposed). In a normal heart, the pulmonary artery is connected to the right ventricle and the aorta is connected to the left ventricle.

In people with double-outlet right ventricle, there is also a hole between the lower heart chambers (ventricles), called a ventricular septal defect (VSD), which can be located in several places in the wall between the ventricles. This causes oxygen-rich blood to flow from the left ventricle to the right ventricle and mix with oxygen-poor blood. Due to this mixing, children born with double-outlet right ventricle can have oxygen saturations that are lower than normal.

Too much blood flow may then be delivered through the pulmonary artery to the lungs, causing heart failure and poor growth over time. In other cases, blood flow through the pulmonary artery may be reduced, which can cause your child's skin to turn a bluish color (cyanosis).

Your child will need surgery to correct this defect and any associated defects. Some children will require surgery in the first few days after birth, and others may have surgery at a few months of age.

Some people may have other associated congenital heart defects, including other holes in the heart (such as an atrial septal defect), heart valve problems or blood vessel problems. The presence of other heart defects may change the medications used and type of surgery performed.