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.
To diagnose double-outlet right ventricle, doctors will generally perform an echocardiogram. Echocardiograms use sound waves to produce an image of the heart. Sound waves bounce off the heart and produce moving images that can be viewed on a video screen.
This test can help doctors to look closely at the heart, including the ventricles, aorta and pulmonary arteries as well as the heart valves. Doctors often use this test to diagnose this condition and any associated defects, and determine the appropriate treatment.
If more information is needed, doctors may conduct cardiac CT and MRI scans, or cardiac catheterization. In cardiac catheterization, your child's doctor inserts a thin, flexible tube (catheter) into an artery or vein in the groin or neck and threads it into the heart. A dye is injected through the catheter to make the heart structures more visible on X-ray pictures. Cardiac catheterization also measures pressure and oxygen levels in the chambers of the heart and in the blood vessels.
Several types of surgery may be performed, depending on the specific heart defect and any associated defects.
Depending on the type of defect, surgeons may:
Create a tunnel (baffle) through the VSD to connect the left ventricle to the aorta
Switch the aorta and pulmonary artery positions, if they are in reversed positions, in order to connect the pulmonary artery to the right ventricle and the aorta to the left ventricle
Insert a patch between the ventricles to close the hole between the ventricles (VSD)
Insert a blood vessel to connect the right ventricle to the pulmonary artery to allow more blood flow if the pulmonary artery is small or absent
Widen the narrowed pulmonary artery to allow more blood flow
Place an aorta to pulmonary artery shunt to provide more blood flow to the pulmonary arteries
Conduct a series of other procedures to allow blood to move to the lungs and for the heart to function with one ventricle, if the defect is complex
Repair any other congenital heart defects, such as atrial septal defects or patent ductus arteriosus
In some infants with inadequate blood flow to their lungs at birth, a temporary procedure may be done to insert a shunt between the aorta and the pulmonary artery. The shunt is then removed later in life during your child's definitive cardiac surgery.
Adults who were born with double-outlet right ventricle will need lifelong care and regular follow-up evaluations from cardiologists trained in evaluating and treating congenital heart conditions (adult congenital cardiologists) to monitor for any changes in their condition. Surgery may need to be performed later in life for valve disease such as narrowing or leakage of the heart valves. Some adults need close monitoring of their aortas and pulmonary arteries, especially if they required surgery early in life. A small group of adults may require medications for treatment of decreased function involving the right or left ventricles.