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Grown-up Congenital Heart Disease (GUCH): Difference between revisions
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Grown-up Congenital Heart Disease (GUCH) (view source)
Revision as of 14:43, 25 January 2012
, 25 January 2012→Transposition of the great arteries
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=== Case report === | === Case report === | ||
=== Introduction === | === Introduction === | ||
[[File:12. TGA.jpg|thumb| | [[File:12. TGA.jpg|thumb|left|Figure 12: Schematic drawing showing transposition of the great arteries. The pulmonary artery is located above the left ventricle (LV) and the aorta is located above the right ventricle (RV).]] | ||
[[File:13. TGA.PNG|thumb|right|Figure 13. Schematic drawing of the circulation in transposition of the great arteries. Left: normal position of the great arteries with the pulmonary and systemic circulation serially connected. Right: transposition of the great arteries with a parallel circulation.]] | |||
Transposition of the great arteries (TGA) accounts for 5-8% of all congenital heart defects and occurs 2-3 times more frequently in males. TGA is best defined as a normal atrioventricular connection with an abnormal ventricular–arterial connection; the morphological left atrium is connected through the left ventricle with the pulmonary artery and the morphological right atrium through the right ventricle with the aorta. (Figure 12)The aorta is often located on the right side and in front of the pulmonary artery (D-TGA). In 70 percent there is an isolated form of TGA, in 30 percent the TGA is accompanied by other heart defects, like VSD or obstruction of the left ventricle outflow tract. | Transposition of the great arteries (TGA) accounts for 5-8% of all congenital heart defects and occurs 2-3 times more frequently in males. TGA is best defined as a normal atrioventricular connection with an abnormal ventricular–arterial connection; the morphological left atrium is connected through the left ventricle with the pulmonary artery and the morphological right atrium through the right ventricle with the aorta. (Figure 12)The aorta is often located on the right side and in front of the pulmonary artery (D-TGA). In 70 percent there is an isolated form of TGA, in 30 percent the TGA is accompanied by other heart defects, like VSD or obstruction of the left ventricle outflow tract. | ||
=== Pathophysiology === | === Pathophysiology === | ||
The circulation in TGA patients is not serial but parallel (figure 13); the venous blood is returned to the systemic circulation through the right atrium and ventricle, while the arterial oxygenated blood is directed back into the pulmonary artery through the left atrium and ventricle. Due to this abnormal circulation there is severe cyanosis directly after birth, therefore it is critical for the ductus arteriosus and foramen ovale to remain open. Without treatment there is a mortality of 30% within one week, 50% within one month and 90% within one year. When an associated VSD is present the chances of survival are higher due to more shunting thus more oxygenated blood in the systemic circulation. These patients are able to reach early adulthood without corrective surgery or intervention. However the pulmonary hypertension that develops in this situation will eventually lead to severe problems. | The circulation in TGA patients is not serial but parallel (figure 13); the venous blood is returned to the systemic circulation through the right atrium and ventricle, while the arterial oxygenated blood is directed back into the pulmonary artery through the left atrium and ventricle. Due to this abnormal circulation there is severe cyanosis directly after birth, therefore it is critical for the ductus arteriosus and foramen ovale to remain open. Without treatment there is a mortality of 30% within one week, 50% within one month and 90% within one year. When an associated VSD is present the chances of survival are higher due to more shunting thus more oxygenated blood in the systemic circulation. These patients are able to reach early adulthood without corrective surgery or intervention. However the pulmonary hypertension that develops in this situation will eventually lead to severe problems. | ||