In Double Outlet Right Ventricle, or DORV, the two Great Arteries (the Aorta and the Pulmonary Artery) are both attached to the right ventricle. This is in contrast to the normal heart, where the Aorta attaches to the left ventricle and the Pulmonary Artery attaches to the right ventricle. Blood from the left ventricle, which would normally leave the heart through the attached aorta, now must cross an abnormal hole in the wall dividing the right and left ventricles, called a Ventricular Septal Defect (VSD), in order to leave the heart and supply the head and body with blood. In this condition, the normally separated oxygen-rich arterial blood and oxygen-poor venous blood is mixed in the right ventricle prior to leaving the heart. Also, the normally low pressure right ventricle can be subjected to increased pressure from the normally high pressure left ventricle.
In most cases, the infant develops symptoms in the first weeks of life – either with “cyanosis” (blue tint to the lips and skin), or with breathlessness and failure to gain weight at a normal rate. There are many variations of this abnormality. Many affected patients also have other defects in the heart or main arteries, including Pulmonary Stenosis (a “tight” pulmonary valve), Pulmonary Atresia (no pulmonary valve) and possibly a coarctation (kink) in the Aorta. Such problems may cause severe obstruction to blood flow and may require urgent cardiac catheterization or surgery.
Treatment, which is surgical, is aimed at repairing the defect and any associated problems, where that can be done. In many cases the heart can be made to function sufficiently well to eliminate symptoms and to allow normal schooling and near normal activities. Most cases are amenable to 'Complete Repair', though more than one operation may be needed.
In a minority of the more complex cases complete repair may not be possible. In such cases treatment aims to make the heart work as effectively as possible. This may involve several operations. For some children the final operation is called the 'Fontan Operation'. This leads to blue blood (low in oxygen) being channelled through the lungs, without any 'pump' driving it. The ventricles then pump the red blood (high in oxygen), round the body. This operation makes the child 'Pink' - but does not correct the original problem.