Double Outlet Right Ventricle (DORV): Causes, Symptoms, Treatment

The left ventricle is the chamber of the heart that normally pumps oxygen-rich (oxygenated) blood to the body, while the right ventricle is the chamber of the heart that pumps deoxygenated blood into the lungs. Normally, both the great arteries, namely the aorta and the pulmonary artery originate from the left ventricle. The aorta exits from the left ventricle and pumps the oxygen-rich blood into the rest of the body. On the other hand, the pulmonary artery exits from the right ventricle and transfers the deoxygenated from the heart into the lungs for the purpose of oxygenation. Double outlet right ventricle (DORV) is a rare congenital heart disorder in which both the great arteries exit from the right ventricle (RV).

In a normal heart structure, the aorta flows out of the LV and the pulmonary artery exits from the RV. But in Double Outlet Right Ventricle (DORV), both arteries flow out of the RV. This becomes problematic as the RV carries oxygen-poor blood which then gets circulated throughout the body. Along with Double Outlet Right Ventricle (DORV), the affected infants generally tend to have a hole in the muscular wall which separates the left ventricle from the right one. This condition is known as ventricular septal defect (VSD). For infants with Double Outlet Right Ventricle (DORV), having a hole in the septum or VSD opening proves helpful as it allows the oxygen enriched blood from the LV to pass into the RV and thereby get pumped into the body together with the oxygen-poor blood. However, even after this the infant’s body does not get its optimal supply of oxygen. Thus, in this situation the heart gets over stressed as it has to work harder to match the body’s oxygen requirement.

The cause of Double Outlet Right Ventricle is still not clear. The severity and symptoms of Double Outlet Right Ventricle depends on the type of DORV. There are four different types of Double Outlet Right Ventricle (DORV). These types are classified based on the location of the VSD in relation with the pulmonary artery and aorta.

Children with Double Outlet Right Ventricle are frequently found to be suffering with other heart defects like pulmonary atresia, pulmonary valve stenosis, endocardial cushion defects, right-sided aortic arch, transposition of the great arteries, coarctation of the aorta, mitral valve issues, etc.

Common symptoms associated with Double Outlet Right Ventricle are bluish colour of the lips and skin (cyanosis), thickening of the nail buds in the toes and hands, difficulty in breathing, swelling in the legs or abdomen, pale skin (pallor), sweating, retarded growth, weight loss, extreme fatigue and poor appetite. Other signs of Double Outlet Right Ventricle (DORV) are rapid breathing and rapid heartbeat (tachycardia), enlarged heart and heart murmur.

Diagnosis of Double Outlet Right Ventricle is made through certain tests and scans like chest x-rays, heart MRI, echocardiogram and cardiac catheterization. Surgery is the best treatment option for Double Outlet Right Ventricle. It aims at closing the hole in the heart and directing blood from the left ventricle into the aorta. Surgery may also be required to move the pulmonary artery or aorta. The number and type of operations the baby or patient would need to undergo in treating Double Outlet Right Ventricle is determined by factors like the type of DORV, presence of other heart problems, severity of the defect and the patient’s overall health condition.

Complications associated with Double Outlet Right Ventricle are heart failure, high blood pressure in the lungs, lung damage and death. Children with Double Outlet Right Ventricle (DORV) may need to have antibiotics before undergoing any dental treatment in order to prevent any infections around the heart. Antibiotics may also be required post surgery for treatment of Double Outlet Right Ventricle (DORV).

The prognosis of the patient and the success of the treatment in Double Outlet Right Ventricle depend on the size of the pumping chambers, location and size of the VSD, location of the aorta and pulmonary artery and the overall health of the patient at the time of diagnosis. Further, presence of other complications like mitral valve problems or coarctation of the aorta, and lung damage due to excessive blood flow to the lungs for a prolonged period greatly impacts the prognosis.

If your child gets tired easily, has bluish lips, faces problems in breathing and has poor growth, contact your doctor immediately. With timely treatment and good medical care, Double Outlet Right Ventricle (DORV) can be dealt with effectively.