What Is Transposition of the Great Arteries?
Transposition of the great arteries (also known as Transposition of the Great Vessels) is a very rare cyanotic congenital heart defect in which the two main arteries that leave the heartare transposed - or reversed.
In this disorder, the aorta originates in the right ventricle and the pulmonary artery originates in the left ventricle.
For people with Transposition of the Great Arteries, this defect changes the way the blood circulates through the body. The blood from the heart that circulates through the body is oxygen-poor, so the body cannot function properly.
Transposition of the great arteries is generally diagnosed within the first few weeks out of the womb and repaired via surgery.
Pathophysiology of Transposition of the Great Vessels
In people without Transposition of the Great Arteries, the pulmonary artery (responsible for carrying blood from the heart to the lungs to become oxygenated) is attached to the right ventricle of the heart.
From the lungs, the freshly oxygenated blood is pumped into the left atrium through the mitral valve into the left ventricle, where the aorta carries the freshly oxygenated blood to the rest of the body.
Circulation in Transposition of the Great Arteries:
As the pulmonary artery and aorta are switched in this congenital heart defect, the pulmonary artery is connected to the left ventricle while the aorta is connected to the right ventricle.
Unoxygenated blood circulates through the right side of the heart and back to the body without passing through the lungs to become oxygenated. Oxygenated blood flows through the left side of the heart and back to the lungs without being circulated through the body.
The circulation of the unoxygenated blood through the body gives the infant a blue tint - making it a cyanotic congenital heart defect.
Risk Factors for Transposition of the Great Vessels:
Most congenital heart defects, like Transposition of the Great Arteries, have no identifiable cause.
Factors that may increase the risk for developing this cyanotic heart defect include:
•Maternal Age over 40
•Family history of Transposition of the Great Vessels or another congenital heart defect
•Poor nutrition during pregnancy
•Male infant
•Chronic Alcoholism
•Poorly controlled maternal (gestational) diabetes
•Rubella infection during pregnancy
•Infant with Down Syndrome
What Are the Symptoms of Transposition of the Great Arteries?
The symptoms that appear in infants with Transposition of the Great Arteries includes:
•Cyanosis - a bluish skin tone
•Shortness of breath
•Failure to thrive
•Lack of appetite
•Clubbing of the fingers and toes
What Are The Complications of Transposition of the Great Vessels?
There are potential complications associated with Transposition of the Great Arteries. In severe cases of this congenital heart defect, it can be fatal if surgery isn't performed.
Other complications include:
•Hypoxia - lack of oxygen to the tissues
•Heart Failure - the heart is unable to pump enough blood to meet the needs of the body
•Lung damage - lack of oxygenated blood can cause lung damage, making breathing hard
After corrective surgery to repair the heart defect, there can be other complications and conditions that appear later in life:
•Narrowing of the coronary arteries (the vessels that supply blood to the heart)
•Leaky heart valves
•Heart Arrhythmias
•Heart muscle weakness, which can lead to heart failure
Diagnosis of Transposition of the Great Vessels:
After a baby is born, the doctor may suspect Transposition of the Great Vessels if the baby has blue-tinged skin or trouble breathing.
If another heart defect is present that allows some oxygen-rich blood travel to the tissues of the body, the cyanosis may not be as evident until later in life.
The following tests may be performed if the doctor suspects Transposition of the Great Arteries:
Chest X-Ray - cannot provide a definitive diagnosis, but allows the doctor to view the size of the heart and the position of the aorta and pulmonary artery.
Echocardiogram - an ultrasound of the heart that allows the doctor to examine the position of the aorta and pulmonary artery, as well as identify any associated defects.
Electrocardiogram - measures the electrical activity of the heart.
Cardiac Catheterization - because it's invasive, this test is reserved for times when other tests cannot provide enough information for a diagnosis. A Cardiac Catheterization measures pressure in the chambers of the heart as well as blood vessels.
Treatment of Transposition of the Great Vessels:
All infants with Transposition of the Great Vessels require surgery to correct this cyanotic heart defect.
Pre-Surgical Treatment:
•Medication - Prostaglandin E1 helps to keep the connection between the aorta and the pulmonary artery open, which can increase blood flow and improve mixing of oxygenated and unoxygenated blood until surgery can be performed.
•Atrial Septostomy - procedure usually performed via cardiac catheterization that enlarges a connection between the heart's upper chambers. This allows for improved mixing of oxygenated and unoxygenated blood.
Surgical Treatment:
•Arterial Switch Operation - this surgical treatment, usually done within the first month of life, is considered to be the gold standard to fix transposition of the great vessels. The arteries are moved into their normal position - pulmonary artery connected to the right ventricle and aorta connected to the left ventricle.
•Atrial Switch Operation - a baffle (or tunnel) is created between the two upper chambers of the heart (the atria), which diverts the unoxygenated blood to the left ventricle and pulmonary artery and the oxygenated blood to the right ventricle and aorta.
Even after surgery, the child will need lifelong care from a cardiologist to monitor his or her heart health.
Tips for Living With Transposition of the Great Vessels:
Caring for a very sick baby is a very scary prospect - it's okay to be afraid.
Let friends and loved ones know specific ways that they can help you.
Read more about how to cope with a chronically ill child.
Being a caregiver can be overwhelming at times - be certain to make some time for yourself.
Read more about caregiving here.
Read more about pediatric caregiving.
Buy a big binder and keep track of all surgeries, medications, diagnoses, procedures. This will help keep your information in one place and keep you on top of your child's health.
Talk about any concerns you have with your doctor. Keep a notebook and write down any questions you might have for your child's care team.
Find a support group in your area for people with diagnoses like your child's. Join it.
Related Resource Pages on Band Back Together:
Congenital heart defects
Caregiving here
How to cope with a chronically ill child
Cyanotic Heart Defects
Pediatric caregiving
Congenital Heart Defect Support Resources (Support Groups):
Mended Little Hearts - support program for parents of children with heart defects and heart disease.
It's My Heart - non-profit organization that provides support to and advocates for those affected by Acquired and Congenital Heart Defects.
Congenital Hearth Information Network provides support, information, and resources.
Adult Congenital Heart Resources:
Adult Congenital Heart Association - a non-profit organization dedicated to improving the quality of life for and extending the lives of adults living with congenital heart defects (CHD).
Congenital Heart Defect Researchers:
Chloe Duyck Memorial Foundation - raises awareness and promotes research of congenital heart disease.
Children's Heart Foundation - funding the most promising research to advance the diagnosis, treatment and prevention of congenital heart defects.
Saving Tiny Hearts Foundation - raising seed money for grossly under-funded, lifesaving grass roots research of congenital heart defects (CHD's)
Other Congenital Heart Defect Resources:
Saving Little Hearts - helps children with congenital heart defects and their families by providing emotional assistance and educational information primarily through the distribution of Care Packages.
International Congenital Heart Foundation - they bring the skills, technology and knowledge to cure and care for children with congenital heart disease to developing countries. ICHF does this regardless of country of origin, race, religion or gender.
Big Love, Little Hearts - is the first and only organization that matches children with CHD who need lifesaving surgery but do not have access to appropriate care, with the many generous organizations, surgeons and healthcare professionals who donate their time, talent and resources to perform these repairs for free.
Preemptive Love - aimed at eradicating backlog of Iraqi children in need of heart surgery in the name of peace between two communities at odds.
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Band Back Together is a group blog created by Aunt Becky from Mommy Wants Vodka for anyone to share their stories. Some of these stories may be hard to read, but they are yours and they are important. Don't be shy, now. Pull up a chair, strap on your leather pants, and let's Band Back Together.
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