 |
|
 |
|
300 Longwood Avenue
Boston, MA 02115
(617) 355-6000
|
|
|
|
|
My Child Has:
|
|
Single ventricle defects
|
|
|
|
|
|
|
Normally, the heart has a right and left ventricle that serve as the heart's pumping chambers. The right ventricle normally pumps blue blood (without oxygen) out of the heart through the pulmonary artery to the lungs for oxygen and the left ventricle normally pumps red blood (with oxygen) through the aorta out of the heart to the body.
Single ventricle defects is an umbrella term used to describe several very different complex congenital (present at birth) heart defects that share the same problem: the heart has only one functional ventricle.
The following congenital heart defects are considered single ventricle defects
|
|
|
- Tricuspid atresia: A defect that occurs when there is abnormal development of the tricuspid valve, which prevents blood from passing from the right atrium to the right ventricle as it should.
- Hypoplastic Left Heart Syndrome: In this syndrome, most of the structures on the left side of the heart, including he left ventricle that pumps blood out of the body, are small and underdeveloped.
- Single Left Ventricle: This occurs when the heart's right ventricle is rudimentary and doesn't perform its job of pumping blood to the lungs. Both atria connect to the single ventricle either through a single valve (this is called a common-inlet ventricle) or separate valves (called double-inlet ventricle). Sometimes it is the left ventricle that is underdeveloped and there is a single right ventricle, but this is rare.
- Mitral valve atresia: The mitral valve separates the left atrium from the left ventricle. When there is mitral valve atresia, the mitral valve does not develop properly and blood from the left atrium does not flow to the left ventricle. When this occurs the left ventricle becomes small and underdeveloped.
- Double outlet right ventricle. Normally, a ventricle has just one outlet. For the left ventricle, it is the aorta. For the right ventricle it is the pulmonary artery that leads to the lungs. In this defect the right ventricle has an outlet to both the aorta and the pulmonary artery. There are usually other heart defects present.
|
|
|
|
The heart forms during the first 8 weeks of fetal development. It is at this time that defects that result in single ventricle 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.
|
|
|
|
|
|
Single ventricle heart defects can cause children to become cyanotic (blue) since a mixture of oxygen-poor (blue) and oxygen-rich (red) blood vessels leaves the heart and goes to the body. Just how much oxygen or how little oxygen depends on a number of factors. Some children will only be mildly cyanotic, while others will not have enough oxygen in the blood to meet the body's needs.
|
|
|
|
|
|
Symptoms are noted shortly after birth. The following are the most common symptoms of single ventricular defects. However, each child may experience symptoms differently. Symptoms may include:
|
|
|
- Blue color of the skin, lips, and nailbeds (cyanosis)
- Rapid breathing
- Labored breathing
- rapid heart rate
- cool, clammy skin
|
|
These symptoms may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.
|
|
|
|
|
|
A pediatric cardiologist and/or a neonatologist may be involved in your child's care. 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. A neonatologist specializes in illnesses affecting newborns, both premature and full-term.
Cyanosis is the major indication that there is a problem with your newborn. Your child's physician may have also heard a heart murmur during a physical examination. A heart murmur is simply a noise caused by the turbulence of blood flowing through the openings that allow the blood to mix.
Other diagnostic tests are needed to help with the diagnosis, and may include the following:
|
|
|
- chest x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film
- electrocardiogram (ECG or EKG) - a test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle stress
- echocardiogram (echo) - a procedure that evaluates 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 catheterization - a 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.
- cardiac magnetic resonance imaging (MRI) - 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
|
|
|
|
The treatments for single ventricle vary by individual depending mostly on the amount of mixing of blood, and how much blood is being pumped through the lungs.
This varies greatly. Babies with too much blood flow to the lungs sometimes receive a pulmonary artery band to restrict blood flow to the lungs. Babies with too little blood flow to the lungs (blue babies) may have a shunt (surgical connection between an artery from the aorta to the pulmonary artery) to increase blood flow to the lungs.
Other procedures may accompany these treatments, depending on the anatomy.
Common to all, however, is that these procedures are temporary in that they stabilize the circulation. Additional procedures are usually necessary as the child grows.
|
|
|
|
|
|
|
|
|
|
|
|
|
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. |
|
|
|
Copyright © Children's Hospital Boston. All rights reserved. |
|
|
|
|
