Complete Transposition of the Great Arteries/TGA or Transposition of Great Vessels: Transposition of the Great Arteries is a very rare congenital heart defect in which the two main arteries leaving the heart are transposed – or reversed. This changes the way the blood circulates through the body. The blood from the heart that circulates through the body does not have enough oxygen. Children with TGA will often have a bluish tint to their skin (cyanosis), clubbing of the fingers and toes (rounded nails), difficulty feeding, and shortness of breath. These symptoms are typically significant enough to be noticed without an intensive medical exam. Doctors will perform a series of exams to determine the proper treatment plan.
Causes: Although some factors, such as rubella or other viral illnesses during pregnancy, maternal age over 40, or maternal diabetes, may increase the risk of this condition, in most cases the cause is unknown.
Possible complications: With TGA, a baby’s tissues will receive too little oxygen. Unless there’s some mixing of oxygen-rich blood and oxygen-poor blood within the baby’s body, he or she won’t be able to survive. Heart failure – a condition in which the heart can’t pump enough blood to meet the body’s needs – may develop over time because the right ventricle is pumping under higher pressure than usual. This added stress may make the muscle of the right ventricle stiff or weak. The lack of oxygenated blood also causes damage to the lungs, making breathing difficult. Even after surgery, complications such as narrowing of the coronary arteries, arrhythmias, weak heart muscles and leaky valves can cause issues.
Treatment: Typically a baby would receive medication to keep a blood vessel called the the ductus arteriosus open, allowing some mixing of the two blood circulations. Treatment is recommended within the first week of life and surgery within the first month, however, many children adopted internationally will not have early intervention, which puts them at greater risk for additional complications.
There are two surgical options used to correct TGA:
- Arterial switch operation. This surgery is used most often use to correct TGA. During this operation, the pulmonary artery and the aorta are moved to their normal positions: The pulmonary artery is connected to the right ventricle, and the aorta is connected to the left ventricle. The coronary arteries also are reattached to the aorta.
- Atrial switch operation. In this surgery, the surgeon makes a tunnel (baffle) between the heart’s two upper chambers (atria). This diverts the oxygen-poor blood to the left ventricle and the pulmonary artery and the oxygen-rich blood to the right ventricle and the aorta. With this procedure, the right ventricle must pump blood to the entire body, instead of just to the lungs as it would do in a normal heart. Possible complications of the atrial switch operation include irregular heartbeats, baffle obstructions or leaks, and heart failure due to problems with right ventricle function. Children adopted internationally are more likely to have had this procedure.
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