What is Double Outlet Right Ventricle?

Double outlet right ventricle is a very rare set of congenital heart defects. Both the pulmonary valve and the aorta are connected to the right ventricle. Children with this condition generally also have a large ventricular septal defect (VSD), and many have extreme pulmonary stenosis.

Even though double outlet right ventricle is complex, newborns may not immediately exhibit problems. In fact, some children can wait a year or so before undergoing any type of repair. The ventricular septal defect is actually the saving grace of these children. It allows for blood to mix between the ventricles, so some oxygenated blood can get to the body.

In other cases, however, the presence of the stenotic pulmonary valve means that not enough blood can get to the lungs for oxygenation. This can result in enlargement of the right ventricle as it attempts to push blood through the narrow passageway of the valve. When pulmonary stenosis is severe, the child may be in heart failure almost immediately after birth and hence require early surgical intervention.

When double outlet right ventricle is not noticed right after birth, as is frequently the case, parents may begin to notice symptoms within a few days, and physicians will ultimately hear the murmur caused by the ventricular septal defect, which may not be audible at first. Symptoms include poor weight gain, failure to thrive, fatigue, poor breathing, blueness at the tips of fingers and toes, and feeding difficulties. If these symptoms exist, contact a pediatrician immediately, as this can be an indication of many different types of heart defects.

Once a heart defect is suspected, the child will be referred to a pediatric cardiologist for testing. Most of these tests are non-invasive, and the most common are chest x-rays and echocardiograms, sonograms of the heart. Cardiologists may also perform a cardiac catheterization, an outpatient procedure which can specifically measure the size of the VSD and the amount of pulmonary stenosis, if any, and thus estimate the best way and time to correct double outlet right ventricle.

When pulmonary stenosis is not present, double outlet right ventricle may be repaired in one operation. The surgeon will repair the ventricular septal defect, but build a conduit through it to connect the left ventricle to the aorta. This approach is called the Rastelli procedure. Other surgeons may opt for the arterial switch operation, commonly also used with transposition of the great arteries, in which the aorta is reconnected to the left ventricle.

If double outlet right ventricle presents with pulmonary stenosis, the pulmonary valve may also require replacement. The new pulmonary valve is either bovine, porcine or donor. It does not grow with the child, and valves generally need replacement every eight to ten years. Double outlet right ventricle can also result in hypertrophy of the left or right ventricles. When these ventricles are too small to allow the heart to work properly, additional surgeries, such as the Fontan, may be employed to manage these defects.

Children with double outlet right ventricle require careful observation before and after surgery. They will probably need to take daily low dose aspirin and antibiotics before dental exams and procedures. Cardiologists may also recommend a few limitations, such as not participating in competitive sports. Though some children will later need valve replacement surgery, the life expectancy of children with this set of defects is quite good, and quality of life is not impaired post-surgically.