Transposition of the Great Arteries

Transposition of the Great Arteries

 

Definition

Transposition of the great arteries is a birth defect causing a fatal condition in which there is a reversal, or switch, in the truncal connections of the two main (great) blood vessels to the heart, the aorta and pulmonary artery.

Description

There are two great arteries, the pulmonary artery and the aorta. Normally, the pulmonary artery carries blood from the right ventricle to the lungs. The aorta carries blood from the left ventricle to the vessels of the rest of the body.
Normally, blood returning to the heart is depleted in oxygen. It goes first to the right atrium of the heart and then to the right ventricle where it is pumped to the lungs. While in the lungs, the blood picks up more oxygen. After the lungs, the blood flows to the left atrium, then the left ventricle, which pumps the blood out through the aorta to the rest of the body, thereby supplying the body with oxygenated blood.
Transposition of the great arteries results in oxygen-depleted blood going to the body. The reason is that the connection of the two great arteries is reversed. In this case, the aorta is connected to the right ventricle. Blood returning to the heart goes to the right atrium and ventricle, which is normal. Then, when the right ventricle pumps the blood out, it goes into the aorta for distribution throughout the body. At the same time, blood in the lungs goes to the left atrium, the left ventricle, but then back to the lungs. This happens because the pulmonary artery is connected to the left ventricle. The result is that highly-oxygenated blood keeps recycling through the lungs, while oxygen-depleted blood recycles through the body without going through the lungs to reoxygenate.
This condition develops during the fetal stage and must be treated promptly after birth if the newborn is to survive. The newborn can survive for a few days because the foramen ovale, a small hole in the septum that separates the two atria, is open, allowing some oxygenated blood to escape and mix into the blood that is being pumped throughout the body. However, the foramen ovale normally closes within a few days after birth.

Causes and symptoms

Transposition of the great arteries is a birth defect that occurs during fetal development. There is no identifiable disease or cause. The main symptom is a "blue" baby appearance, caused by a general lack of oxygen in the body's tissues.

Diagnosis

Diagnosis is made immediately after birth, when it is observed that the newborn is lacking oxygen. This is noted by the bluish color of the newborn, indicating cyanosis, a lack of oxygen. A definite diagnosis is made by x ray, electrocardiography (ECG), and echocardiography.

Treatment

The only treatment for this condition is prompt heart surgery shortly after birth. In surgery, the two great arteries are reconnected to their proper destination. This restores the normal blood flow pattern. The coronary arteries are also reconnected, so that they can supply blood to the heart itself. A catheter may be used to maintain or enlarge the opening between the two atria until surgery can be performed.

Prognosis

Left untreated, this disease is fatal within the first weeks of life.

Prevention

Because there is no identifiable cause, there is no way to prevent this condition.

Resources

Books

Alexander, R. W., R. C. Schlant, and V. Fuster, editors. The Heart. 9th ed. New York: McGraw-Hill, 1998.

corrected transposition of the great vessels

(1) Anatomically corrected malposition of the great arteries—more popularly termed transposition of the great arteries.
(2) Physiologically corrected transposition of the great arteries.
References in periodicals archive ?
Shane was born five weeks prematurely, weighing only 4lb, and was diagnosed with transposition of the great arteries, which means the pumps in his heart were the wrong way round, and he also had a hole in the heart.
Sarah was suffering from transposition of the great arteries, which meant that she needed major heart surgery because her heart was not working properly.
The tot, who is now 10 weeks old, was born with a rare condition called transposition of the great arteries.
The other associated cardiac anomalies include left coronary artery originating from the pulmonary trunk, coronary arterioventricular fistulae, right coronnary artery atresia, pulmonar artery atresia, subpulmonary stenosis, anomalous pulmonary venous return, patent ductus arteriosus, tetralogy of Fallot, transposition of the great arteries, dextrocardia and aortic atresia (8,9).
This year the conference focus is on developing treatment guidelines for three common heart defects: transposition of the great arteries, teratology of Fallot and single ventricle lesions.
Detection of transposition of the great arteries in fetuses reduces neonatal morbidity and mortality.
Transposition of the great arteries, where the positions of the two major arteries leaving the heart are reversed, so that each arises from the wrong pumping chamber.
Lucas has a heart condition - congenitally corrected transposition of the great arteries - but it doesn't cause him any problems and means he needs to be monitored annually.
He was then taken to Cardiff's University Hospital of Wales where he was diagnosed with transposition of the great arteries and a single coronary artery.
Transposition of the great arteries (TGA) is an uncommon congenital heart disease with a reported incidence of 2.
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