Double outlet right ventricle (DORV) is a very rare congenital heart defect in which both of the great arteries of the heart (the aorta and the pulmonary artery) are connected to the right ventricle instead of separate ventricles. The degree of the defect can vary.

DORV patients almost always have a ventricular septal defect (VSD), which is a hole in the wall (septum) between the two pumping chambers of the heart, the left and right ventricles. With DORV, the VSD actually helps patients because it allows oxygen-rich blood to pass from the left ventricle to the right, where it is pumped into the aorta and to the rest of the body. Circulation of oxygen-rich blood is accomplished with the VSD but only at great stress to the system. In addition, the oxygen levels in the blood are not as high as those found in patients with normal hearts.

Since DORV is a congenital heart defect, it is established during the fetal development – during the first eight weeks when the heart is formed. Congenital heart defects may be due to a genetic link, such as a chromosome abnormality or a gene defect. They also may be caused by exposure to environmental toxins. In most cases, however, heart defects, such as DORV, occur by chance with no definitive reason, as yet, for their occurrence.

The signs and symptoms of DORV usually appear in infancy. They will vary depending on the position and size of the VSD. The most common symptoms include a heart murmur, rapid breathing, fatigue and sweating. In addition, an infant or child may demonstrate a bluish-tint to the skin (cyanosis) and have poor weight gain. The signs and symptoms of DORV, however, may be similar to other medical conditions or heart problems. If a child demonstrates these symptoms, a physician should evaluate him or her.

An infant or child will likely be referred to a pediatric cardiologist for the definitive diagnosis of DORV. A physical examination and various tests, such as an echocardiogram, will be used to diagnose DORV defects.

Surgery is necessary to repair the heart defects from DORV. There are several surgical options and the specific treatment will be based on the extent of the condition and medical considerations of the child. The outlook for recovery for most patients undergoing treatment for DORV is very good. A pediatric cardiologist, however, must follow the patients to monitor the presence of any heart difficulties.  Although DORV may be corrected, it may not be cured and care by specialists dealing with congenital heart disease will be necessary indefinitely.