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| Tetralogy of Fallot : |
Tetralogy of Fallot is an uncommon condition consisting of four ("tetra") defects within the heart structures of infants and young children. Together these defects cause reduced blood flow to the lungs and mixing of blood from both sides of the heart. This results in oxygen-poor blood flowing out of the heart and into the body. Consequently, infants and children with tetralogy of Fallot usually have blue-tinged skin (cyanosis).
Tetralogy of Fallot, which is present at birth, is usually diagnosed during infancy. However, it may not be detected until later in life, depending on the severity of the heart defects and symptoms. Tetralogy of Fallot occurs in about five out of every 10,000 babies and affects males and females equally. With early diagnosis followed by corrective surgery and ongoing care, the majority of children with tetralogy of Fallot survive to adulthood. |
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| Treatment |
Most babies with tetralogy of Fallot need a type of surgery called intracardiac repair, which is typically performed during the first year of life. During this procedure, the surgeon places a patch over the ventricular septal defect to close the hole between the ventricles. He or she also repairs the narrowed pulmonary valve and widens the pulmonary arteries to increase blood flow to the lungs. After intracardiac repair, the oxygen level in the blood increases and your baby's symptoms will lessen.
Occasionally babies need to undergo a temporary surgery before having intracardiac repair. If your baby was born prematurely or has pulmonary arteries that are underdeveloped (hypoplastic), doctors will create a bypass (shunt) between the aorta and pulmonary artery. This bypass increases blood flow to the lungs. When your baby is ready for a full repair, the shunt will be removed. |
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atrioventricular canal (AVC or AV canal) - atrioventricular canal is a complex heart problem that involves several abnormalities of structures inside the heart, including atrial septal defect, ventricular septal defect, and improperly formed mitral and/or tricuspid valves. |
| Atrioventricular Canal Defect |
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| Some of the problems that cause too little blood to pass through the lungs include the following: |
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tricuspid atresia (TA) - in this condition, there is no tricuspid valve, therefore, no blood flows from the right atrium to the right ventricle. Tricuspid atresia defect is characterized by the following: |
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a small right ventricle |
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a normal left ventricle |
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diminished pulmonary circulation |
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cyanosis - bluish color of the skin and mucous membranes caused from a lack of oxygen. |
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A surgical shunting procedure is often necessary to increase the blood flow to the lungs. |
Anatomy of a heart
with tricuspid atresia |
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pulmonary atresia (PA) - a complicated congenital defect in which there is abnormal development of the pulmonary valve. Normally, the pulmonary valve is found between the right ventricle and the pulmonary artery. It has three leaflets that function like a one-way door, allowing blood to flow forward into the pulmonary artery, but not backward into the right ventricle.
With pulmonary atresia, problems with valve development prevent the leaflets from opening, therefore, blood cannot flow forward from the right ventricle to the lungs. |
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transposition of the great arteries (TGA) - with this congenital heart defect, the positions of the pulmonary artery and the aorta are reversed, thus:
• the aorta originates from the right ventricle, so most of the blood returning to the heart from the body is pumped back out without first going to the lungs.
• the pulmonary artery originates from the left ventricle, so that most of the blood returning from the lungs goes back to the lungs again |
Anatomy of a heart with
transposition of the
great arteries |
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double outlet right ventricle (DORV) - a congenital heart defect (one that occurs as the heart is forming during pregnancy) in which both the aorta and the pulmonary artery are connected to the right ventricle. |
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truncus arteriosus - the aorta and pulmonary artery start as a single blood vessel, which eventually divides and becomes two separate arteries. Truncus arteriosus occurs when the single great vessel fails to separate completely, leaving a connection between the aorta and pulmonary artery. |
Anatomy of a heart with
truncus arteriosus |
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