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Double Outlet Right Ventricle (DORV)
What is a Double Outlet Right Ventricle?
A double outlet right ventricle (DORV) is a congenital heart disease in which the aorta rises from the right ventricle, instead of from the left ventricle. Both the pulmonary artery and aorta come from the same pumping chamber. No arteries arise from the left ventricle(Figure: A). Normally, only the pulmonary artery that carries blood to the lungs for oxygen arises from the right ventricle. The aorta, which carries oxygenated blood from the heart to the body, normally arises from the left ventricle.
 Figure: A
This defect almost always coincides with a ventricular septal defect (VSD) an abnormal opening in the septum (wall that separates the two ventricles) that allows blood to pass between the right and left ventricles. In the case of DORV, a ventricular septal defect is helpful because it allows the oxygen rich blood of the left ventricle passage to the right ventricle, which pumps it to the aorta and out of the heart to the body. If this passage didn't exist, there would be no way for oxygen-rich blood to get to the aorta. Still, this oxygen rich blood mixes with oxygen poor blood, so oxygen levels in the blood are not optimal, and the heart must work extra hard to maintain circulation.
What are the differnet types of Double Outlet Right Ventricle defects?
Several variations of DORV exist. Four types are classified by the position of the ventricular septal defect with respect to the great arteries. These include: - DORV with subaortic ventricular septal defect: the VSD is located just below the aorta.
- DORV with subpulmonary VSD: the VSD occurs below the pulmonary artery.
- DORV with doubly committed VSD: the VSD occurs in two places, both below the aorta and the pulmonary artery.
- DORV with non-committed VSD: the VSD occurs in a position that is away from either of the great arteries.
What are the symptoms of Double Outlet Right Ventricle?
Several variations of DORV exist. Four types are classified by the position of the ventricular septal defect with respect to the great arteries. These include: - Fatigue
- Sweating
- Heart Murmur
- Poor weight Gain
- Rapid Breathing
- Congested Breathing
- Shortness of Breath
- Blue color of the skin, lips and nailbeds (cyanosis)
- Baby tires easily, especially when feeding
What treatments are available for Double Outlet Right Ventricle?
The treatment of choice is typically surgery that aims to connect the aorta to the left ventricle. This can be done through several different approaches that vary depending on your child's circumstance.
One method is known as intraventricular repair, meaning that the repair occurs entirely within the right ventricle. Pediatric cardiac surgeons create a kind of tunnel known as a baffle that connects the left ventricle to the aorta through the VSD. This way, blood flows from the left ventricle to the aorta.
Another procedure is called an arterial switch operation and it involves just that, switching the positions of the great arteries. There are two steps involved. During the first step, a tunnel is created from the VSD to the pulmonary artery, connecting the left ventricle to the pulmonary artery. So now, with the right ventricle connected to the aorta, the connections between the great ventricles and great vessels are completely in reverse of what they should be. The second step involves disconnecting and reconnecting the vessels to their proper ventricles. The coronary arteries must be transferred to the newly positioned aorta as well, or "blue" blood will supply the muscle of the heart. Associated holes between the chambers of the heart are also closed.
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The information provided is for educational purposes only and is not intended to be used as a substitute for an informed discussion with your physician. If you have further questions regarding this procedure, please write them down so your physician or other healthcare professionals can answer them for you. | |
