Double Outlet Right Ventricle

Double outlet right ventricle (DORV) is a congenital heart defect in which both of the great arteries (the pulmonary artery and the aorta) connect to the right ventricle.

About Double Outlet Right Ventricle

In a normal heart, the pulmonary artery connects to the right ventricle (responsible for pumping oxygen-poor blood through the pulmonary artery and to the lungs) and the aorta connects to the left ventricle (responsible for pumping oxygen-rich blood through the aorta and to the rest of the body). Double outlet right ventricle (DORV) is diagnosed when both the pulmonary artery and the aorta connect to the right ventricle.

A hole between the two pumping chambers of the heart (ventricular septal defect) is always present in patients with DORV. This allows the mixing of oxygen-rich and oxygen-poor blood and causes the blood to not be as oxygenated as it should be.

There are 4 types of DORV, with each type classified according to where the ventricular septal defect is located in relation to the great arteries:

• Double outlet right ventricle with a doubly committed ventricular septal defect occurs when there are 2 ventricular septal defects–one below the aorta and one below the pulmonary artery.
• Double outlet right ventricle with a subaortic ventricular septal defect occurs when the ventricular septal defect is located just below the aorta.
• Double outlet right ventricle with a subpulmonary ventricular septal defect occurs when the ventricular septal defect is located below the pulmonary artery. This type of DORV is also called Taussig-Bing anomaly.
• Double outlet right ventricle with a non-committed (or remote) ventricular septal defect occurs when the ventricular septal defect is not located near the aorta or the pulmonary artery.

Symptoms of Double Outlet Right Ventricle

While some forms of DORV result in too much blood flow to the lungs, which causes damage to the heart and lungs and, over time, can cause heart failure, other forms of DORV result in not enough blood flow to the lungs. Some children with DOVR may have an evenly balanced amount of blood flowing to the lungs and body and may not experience symptoms at all.

Symptoms of DORV may include:

• Difficulty feeding
• Fast breathing
• Fatigue
• Poor weight gain
• Sweating during feeds
• Skin that appears pale or blue in color (cyanosis)

Diagnosing Double Outlet Right Ventricle

During physical examinations, your child’s doctor listens to your child’s heart and lungs and may detect a heart murmur, which are extra sounds heard throughout the cardiac cycle due to increased blood flow. If your pediatrician suspects increased blood flow, a recommendation to see a pediatric cardiologist may be made. Your doctor may also refer you to a pediatric cardiologist if your child is breathing fast or has poor exercise tolerance (for babies, this includes poor feeding).

Tests performed when diagnosing DORV may include:

• Cardiac Catheterization: During cardiac catheterization, a small catheter (thin tube) is inserted into a larger blood vessel, typically in the groin, and guided to the heart where blood pressure and oxygen measurements can be taken in the aorta and pulmonary artery as well as the four chambers of the heart. A dye can also be injected through the tube to make the heart’s structure more visible on an X-ray.
• Cardiac MRI or CT Scan: A cardiac MRI or CT scan is used to take more detailed images of the heart to help define the anatomy and detect anomalies.
• Chest X-Ray: A chest X-ray produces an image of the tissue and bones in the heart and lungs and helps your provider assess the shape, size, and structure of the heart and lungs as well as the aeration of or any congestion in the lungs.
• Echocardiogram: An echocardiogram uses ultrasound technology to create a moving image of the heart and its valves, allowing your provider to assess the structure and function of the heart. An echocardiogram also helps provide information about blood flow and how well the heart is pumping blood.
• Electrocardiogram (ECG or EKG): An electrocardiogram uses electrodes that are placed on the body to record the electrical activity taking place in the heart. An ECG/EKG test helps detect abnormal rhythms, such as cardiac arrhythmias, stress on the heart, and damage to the heart muscles.

Treating Double Outlet Right Ventricle

While medications can be used to help children, who have too much blood going to their lungs, feel better and grow larger in size, surgery is needed to repair the defect and connect the great arteries with their correct pumping chambers. Depending upon the severity of the defect and any other associated defects, the timing of surgery may be urgent, or the surgery may be able to be performed electively at a later time.

Treatment options for DORV may include:

• Arterial Switch: A type of surgery in which the position of the arteries are reversed so that the pulmonary artery is connected to the right ventricle and the aorta is connected to the left ventricle. If the child also has a ventricular septal defect, this is typically closed by placing a patch over the hole during the same operation.
• Intraventricular Repair: A type of surgery in which a tunnel (baffle) is created through the ventricular septal defect to connect the aorta to the left ventricle.

Care Team Approach

The Texas Center for Pediatric and Congenital Heart Disease, a clinical partnership between Dell Children’s Medical Center and UT Health Austin, takes a multidisciplinary approach to your child’s care. This means your child and your family will benefit from the expertise of multiple specialists across a variety of disciplines. Your care team will include pediatric cardiologists, cardiothoracic surgeons, interventional cardiologists, critical care specialists, hospitalists, anesthesiologists, perfusionists, nurses, advanced practice providers, social workers, psychologists, child life specialists, dietitians, physical and occupational therapists, pharmacists, and more, who work together to provide unparalleled care for patients every step of the way. We collaborate with our colleagues at the Dell Medical School and The University of Texas at Austin to utilize the latest research, diagnostic, and treatment techniques, allowing us to identify new therapies to improve treatment outcomes. We are committed to communicating and coordinating your child’s care with referring physicians and other partners in the community to ensure that we are providing comprehensive, whole-person care.

Learn More About Your Care Team