Double-outlet right ventricle (DORV) is a rare heart defect. In a normal heart, the blood flows in from the body to the right atrium. It then goes into the right ventricle. Next, the blood travels to the lungs through the pulmonary valve. Here, it picks up fresh oxygen. The blood returns to the left atrium and goes into the left ventricle. The blood moves out to the rest of the body.
Normally, the aorta, which is the largest artery in the body, comes from the heart’s left ventricle. But when DORV occurs, the aorta and the pulmonary artery are attached to the right ventricle.
Another heart defect called ventricular septal defect (VSD) usually occurs with DORV. VSD is a hole in the wall between the right and left ventricle. DORV can be categorized into several types depending on the position of the VSD.
Along with these conditions, the pulmonary valve may be narrowed. DORV is a serious condition that requires care right away.
For many heart defects, the risk factors are unclear. Some risk factors for DORV may include:
Symptoms may vary depending on where the VSD is located. Symptoms may include:
You will be asked about your child’s symptoms and medical history. A physical exam will be done. During the exam, the doctor may also detect a heart murmur.
Images may be taken of your child's bodily structures. This can be done with:
Your child's heart activity may be measured. This can be done with electrocardiogram (EKG).
Talk with the doctor about the best treatment plan for your child. Often, surgery is needed right away. Treatment options include:
Surgery can be done to correct DORV. The goal of surgery is to connect the aorta to the left ventricle. Surgery can range in complexity. The doctor may insert a shunt or create a new tunnel to connect the left ventricle to the aorta through the VSD. Pulmonary artery banding may be used to limit blood flow to the lungs. Depending on other abnormalities, a more complex surgery may be needed to change the position of the large arteries and reconnect other vessels.
American Heart Association
Healthy Children—American Academy of Pediatrics
Canadian Cardiovascular Society
Heart and Stroke Foundation of Canada
Congenital ventricular septal defect (VSD) in children and adults. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T116076/Congenital-ventricular-septal-defect-VSD-in-children-and-adults. Updated January 25, 2016. Accessed June 6, 2016.
Double-outlet right ventricle in children. Boston Children's Hospital website. Available at: http://www.childrenshospital.org/conditions-and-treatments/conditions/double-outlet-right-ventricle-dorv. Published 2010. Accessed June 6, 2016.
Transposition of the great vessels. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T115174/Transposition-of-the-great-vessels. Updated January 25, 2016. Accessed June 6, 2016.
Last reviewed June 2016 by Karri Kassir, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.