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Complete Transposition of the Great Arteries
Tuesday, 18 November 2003
Sunday, 28 November 2004
D-TGA. L-TGA

What

Some babies can be born with a heart disorder called Transposition of the Great Arteries (TGA). The blood vessels that normally deliver blood to the body, send blood to the lungs instead, while the vessels that should deliver blood to the lungs, deliver the blood to the body. This results in blood without oxygen being sent to the tissues of the body. Babies can survive for a time with this because they have other blood vessel abnormalities that cause oxygen rich blood to mix with the oxygen poor blood, thus providing at least some oxygen to the body.

Who

Complete TGA occurs in about 5-7% of all congenital heart defects and is the most common form of cyanotic (oxygen-deprived blue color) heart disease. It is three times more common in males than in females. As a whole, the incidence of congenital heart defects is 8 per 1000 live births.

Signs and Symptoms

Infants with TGA have symptoms starting shortly after birth that include: -Blue color to the skin (cyanosis) that does not improve with oxygen. -Signs that the heart cannot pump enough blood for the body’s needs (congestive heart failure): -Poor feeding -Sweating -Poor weight gain -Fast or labored breathing -Enlarged liver -Heart murmur (heart sounds of blood passing through abnormal holes in the heart) The severity of symptoms depends on how much mixing there is between blood that has oxygen and blood that does not.

Possible Causes

The exact cause of this defect is still unknown.

Diagnosis

Making the diagnosis of complete TGA involves an ultrasound of the heart (echocardiogram) that will provide key anatomical information. Often, infants with TGA will have other heart anomalies that are important to identify because it will influence the type of corrective surgery performed. Blood tests, which include a blood gas, blood electrolytes, and complete blood count, are routinely done because the infants are usually critically ill shortly after birth. Oxygen will be used to try to improve the child’s blue color. Chest x-rays and EKG (electrocardiogram) may also be performed to identify characteristic abnormalities. Once the exact nature of the problem is identified, a pediatric cardiologist will work with a cardiothoracic surgeon to plan the best corrective surgical procedure.

Treatment

Before any surgeries are performed, disorders in blood electrolyte levels and excessive accumulation of acid in the blood are corrected. A medication known as PGE (prostaglandin) will be started to keep the patent ductus arteriosus (PDA) open. The PDA is a short blood vessel that passes blood from the unborn baby’s heart to the aorta or vessel that delivers blood to the body. When the baby is born and breathes air, the PDA is supposed to close down. If the PDA can be kept open in an infant born with TGA, it allows oxygen rich blood to get to the body. A cardiac catheterization procedure is usually performed prior to any corrective open-heart surgery. The catheterization is performed under anesthesia and involves the insertion of a tube through the groin into the vessels that lead to the heart. Once the catheter is in proper position, the cardiologist will use it to create an artificial hole between the right and left sides of the heart, allowing more mixing of oxygen poor and rich blood. This procedure will give the surgeons time to plan a corrective open-heart surgery. While the exact surgery performed will depend on each child’s anatomy, the most commonly performed surgery is the arterial switch (named Jatene procedure). The arterial switch is usually performed in the first 2-4 weeks of life and involves the cutting of the aorta and main pulmonary artery followed by sewing them onto their anatomically correct locations.

Prognosis

Without surgical correction, the natural course is progressive congestive heart failure leading to death before the age of 6 months. The overall 5-year survival after the arterial switch operation is more than 80%. Children must be monitored closely after the surgery for leaky or narrowed heart valves. Other potential complications include heart attacks and rarely irregular heart rhythms. Children with more complicated heart problems requiring higher risk surgeries are at greater risk for complications.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

University of Michigan congenital Heart Disease Webpage
Clearly written description of the condition and recommendations for parents.

The Children s Heart Institute
Nice graphical illustration of anatomy and good links to related topics.

Royal Children s Hospital Website
Nice illustrations of different surgeries performed and printable handouts in pdf format

NIH Medline Website
More technical description of the condition from the NIH.

Congenital Heart Information Network
Parent based support group web-page Yahoo group site for parents

http://health.groups.yahoo.com/group/L-TGA/
Open forum to share experiences and get support

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Google Search for Complete Transposition of the Great Arteries

References and Sources

Emedicine.com article by John R. Charpie http://www.emedicine.com/ped/topic2548.htm Park, M. Pediatric Cardiology for Practitioners. 4th edition pp. 116-119.