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300 Longwood Avenue
Boston, MA 02115
(617) 355-6000
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My Child Has:
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Double Outlet Right Ventricle
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A DORV is a very rare congenital heart defect in which both the pulmonary artery and the aorta known as the great vessels -- arise from the right ventricle. 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.
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 didnt 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.
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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:
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- 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.
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The heart forms during the first 8 weeks of fetal development. It is at this time that defects such as DORV emerge. Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more frequently in certain families. Most of the time, these kinds of heart defects occur sporadically (by chance), which no clear reason for their development.
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The position the ventricular septal opening will affect the type of symptoms noted, the severity of symptoms, and the age at which they first occur. The following are the most common symptoms of DORV defects. However, each child may experience symptoms differently. Symptoms include:
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- Fatigue
- Sweating
- Heart murmur
- Rapid breathing
- Congested breathing
- Shortness of breath
- Blue color of the skin, lips and nailbeds (cyanosis)
- Disinterest in feeding or tiring while feeding
- Poor weight gain
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The symptoms of DORV may resemble other medical conditions or heart problems. Always consult your childs physician for a diagnosis.
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If your child is experiencing any symptoms mentioned above or your newborn baby was born with a bluish tint to the skin, he or she may be referred to a pediatric cardiologist. A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. The cardiologist will perform a physical examination, listening to the heart and lungs, and making other observations that help with the diagnosis.
The doctor will also investigate a heart murmur, which is simply a noise heard through the stethoscope, caused by the turbulence of blood flow. The location in the chest that the murmur is heard best, as well as the loudness and quality of the murmur (harsh, blowing, etc.) will give the cardiologist an initial idea of the kind of heart problem your child may have. Some combination of the following medical tests are also used to diagnose DORV defects:
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- Echocardiogram This test is used to evaluate the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves.
- Cardiac Magnetic Resonance Imaging a non-invasive test that uses three-dimensional imaging technology produced by magnets to accurately determine blood flow and functioning of the heart as it is working.
- Cardiac catheterization A more invasive procedure that gives very detailed information about the structures inside the heart. Under sedation, a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin, and guided to the inside of the heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta. Contrast dye is also injected to more clearly visualize the structures inside the heart.
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Specific treatment for DORV will be determined by your childs physician based on:
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- Your childs age, overall health and medical history
- Extent of the condition
- Your childs tolerance for specific medications, procedures, or therapies
- How your childs doctor expects the condition may progress
- Your opinion or preference
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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 childs 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 on this website should not be taken as medical advice,
which can only be given to you by your personal health care professional. |
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Copyright © Children's Hospital Boston. All rights reserved. |
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