Transposition of the great vessels overview
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Transposition of the great vessels Microchapters |
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Classification |
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Differentiating Transposition of the great vessels from other Diseases |
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Diagnosis |
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Treatment |
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Surgery |
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Transposition of the great vessels overview On the Web |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editors-In-Chief: Priyamvada Singh, MBBS [2]; Cafer Zorkun, M.D., Ph.D. [3]; Keri Shafer, M.D. [4]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [5]
Overview
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Transposition of the great vessels (TGV) is a group of congenital heart defects (CHDs) involving an abnormal spatial arrangement of any of the primary blood vessels: superior and/or inferior vena cavae (SVC, IVC), pulmonary artery, pulmonary veins, and aorta. CHDs involving only the primary arteries (pulmonary artery and aorta) belong to a sub-group called transposition of the great arteries (TGA). The clinical signs and symptoms associated with TGV may range from a change in blood pressure to an interruption in circulation, depending on the nature and degree of the misplacement and which vessels are involved.
The term "TGV" is often used as a more specific reference to transposition of the great arteries TGA; however, TGA only relates to the aorta and the pulmonary artery, whereas TGV is a broader term which can relate to these vessels as well as the SVC, IVC, and pulmonary veins. In its strictest sense, transposition of vessels relates only to defects in which two or more vessels have "swapped" positions; in a broader sense, it may be taken to relate to any defect in which a vessel is in an abnormal position.
Although "transposed" literally means "swapped", many types of TGV involve vessels that are in abnormal positions, while not actually being swapped with each other. The terms TGV and TGA are most commonly used in reference to dextro-TGA (d-TGA) - in which the arteries are in swapped positions; however, both terms are also commonly used, though to a slightly lesser extent, in reference to levo-TGA (l-TGA) - in which both the arteries and the ventricles are swapped; while other defects in this category are almost never referred to by either of these terms. When no other heart defects are present it is called Simple TGV; when other defects are present it is called Complex TGV.
In TGA, the hallmark is ventriculoarterial discordance, in which the aorta (the main artery that carries blood to the body) arises from the morphologic right ventricle and the pulmonary artery (the artery that carries low oxygen blood to the lungs) arises from the morphologic left ventricle. This results in two separate and parallel circulations, which must communicate between them in order to survive after birth.
Most patients have an interatrial communication. Two-thirds have a patent ductus arteriosus, and about one-third have a ventricular septal defect VSD. Stenosis, or other defects, of valves and/or vessels may also be present.