Double-outlet right ventricle is a heart condition present at birth. That means it's a congenital heart defect. In this condition, the body's main artery and the lung artery do not connect to the usual areas in the heart. The body's main artery is called the aorta. The lung artery is called the pulmonary artery.
Sometimes these blood vessels also are reversed from their usual positions.
In a typical heart, the aorta connects to the left lower heart chamber. The pulmonary artery connects to the right lower heart chamber.
In babies with double-outlet right ventricle, both the aorta and the pulmonary artery connect partially or completely to the right lower heart chamber.
Babies with double-outlet right ventricle also have a hole between the lower heart chambers. The lower heart chambers are called the ventricles. The hole is called a ventricular septal defect. The hole causes oxygen-rich blood to mix with oxygen-poor blood. Babies with this condition may not get enough oxygen in the bloodstream. Their skin may appear gray or blue.
Double-outlet right ventricle may occur with other heart problems present at birth. These problems may include other holes in the heart, heart valve problems or blood vessel problems.
If too much blood flows through the pulmonary artery to the lungs, it can lead to heart failure and poor growth.
A test called an echocardiogram can diagnose double-outlet right ventricle. The test uses sound waves to create images of the beating heart. It can show blood flow through the heart and heart valves.
If more details about the heart are needed, other tests may be done. Tests to check the heart may include:
Some babies with double-outlet right ventricle need heart repair surgery within the first few days of birth. Others may have surgery at a few months of age.
More than one type of surgery may be done. The type of surgery depends on the specific heart problems.
The heart surgeon may do one or more of the following:
In some newborn babies, a temporary procedure using a tube called a shunt may be done to increase blood flow to the lungs. The heart surgeon places the shunt between the baby's aorta and the pulmonary artery. The shunt is removed later in life during heart surgery to repair the double-outlet right ventricle.
A person born with double-outlet right ventricle needs regular health checkups for life. Adults should see a health care provider trained in evaluating and treating congenital heart conditions. This type of provider is called an adult congenital cardiologist.
Later in life, surgery may be needed if a heart valve is narrowed or leaks blood backward.
Some adults born with double-outlet right ventricle need medicine to help the right or left lower heart chambers work better.
