Transposition of the great vessels

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Transposition of the great vessels

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A 3-day-old female infant is brought to the emergency department due to difficulty breathing. She was born at term through normal vaginal delivery to a 30-year-old woman who had full prenatal care. The mother reports that the patient was fine after birth and recently started developing blue-colored skin and rapid breathing at home. Temperature is 37°C (98.6°F), pulse is 155/min, respirations are 65/min, and blood pressure is 80/50 mm Hg. Pulse oximetry shows an oxygen saturation of 80% on room air. Arterial blood gas analysis on room air shows PO2 of 30 mm Hg. Physical examination shows central cyanosis and retractions of the rib cage. 100% oxygen through a nasal cannula and PGE1 are administered. The patient’s airway is stable, and a chest radiograph is obtained (shown below). Which of the following is the most likely diagnosis?  


Reproduced from: Radiopaedia 

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Transposition of great vessels p. 304

cyanosis with p. 728

embryologic development p. 288

maternal diabetes and p. 306

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Normally, the heart is set up so that the left ventricle pumps oxygenated blood out to the body through the aorta; deoxygenated blood comes back to the right atrium, flows into the right ventricle, and is pumped to the lungs through the pulmonary artery. From the pulmonary artery, it comes back to the left atrium, flows into the left ventricle, and the whole process restarts. The “great arteries,” are the two main arteries taking blood away from the heart: the aorta and pulmonary artery. “Transposing” means that two things switch places with each other. So, transposition of the great arteries, or TGA, is when these two arteries swap locations.

Normally, blood flows through all of these chambers and blood vessels in a big circuit, but if you switch these two main arteries, you switch from one big circuit to two smaller circuits. On the left side, blood is now pumped from the left ventricle, to the pulmonary artery, and to the lungs; it then comes back to the left atrium and left ventricle, and restarts the circuit. On the right side, blood is pumped out of the right ventricle through the aorta, and then goes to the body; blood comes back the the right atrium and right ventricle, and restarts the circuit. Blood on the right side therefore never gets oxygenated, and blood on the left side never gets deoxygenated. This isn’t good. This situation is actually called complete TGA, or sometimes dextro-TGA or d-TGA; dextro means “right,” because, in this case, the aorta is in front of and primarily to the right of the pulmonary artery.

Summary

Transposition of the great vessels (TGA) is a congenital heart defect in which the positions of the two main blood vessels leaving the heart, the pulmonary artery and the aorta, are switched. This means that the oxygen-rich blood from the lungs is pumped back to the lungs instead of being circulated to the body. The oxygen-poor blood from the body is pumped back to the body instead of being circulated to the lungs. TGA can cause severe oxygen deprivation in the body and can lead to life-threatening complications. TGA is not compatible with life unless there is a shunt (VSD, PDA, patent foramen ovale). Also, without prompt treatment, most infants die within the first few months of life. The treatment for TGA involves surgery to switch the positions of the blood vessels.

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