Overview

Double-outlet right ventricle is a heart condition present at birth (congenital) in which two large blood vessels don't connect to the heart normally. In infants born with this condition, 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) connect partially or completely to the right lower heart chamber (ventricle). Sometimes these blood vessels are also reversed from their normal positions.

In a normal heart, the aorta connects to the left ventricle and the pulmonary artery connects to the right ventricle.

In infants with double-outlet right ventricle, there's also a hole between the right and left ventricles (ventricular septal defect). This causes oxygen-rich blood to mix with oxygen-poor blood. Children born with double-outlet right ventricle can have a lower than normal amount of oxygen in the bloodstream.

If too much blood flows through the pulmonary artery to the lungs, it can cause 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.

Some children will need surgery within the first few days of birth to correct the heart defect. Others may have surgery at a few months of age.

Also, some people with double-outlet right ventricle may have other associated congenital heart defects, including other holes in the heart, heart valve problems or blood vessel problems.