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Transposition of the Great Arteries: Grace's Story

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What is Transposition of The Great Arteries (TGA)?

CHOP cardiologists describe how blood flows through a normal heart, and what is different when a child has TGA.

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Transposition of the Great Arteries (TGA) is a severe congenital condition in which the body’s largest arteries are incorrectly attached to the chambers of the heart. In this 23-minute video, you'll learn how experts from the Fetal Heart Program and Cardiac Center identify TGA during pregnancy, support the baby's delivery, and provide surgery and comprehensive care after birth.

Transcript: What is Transposition of The Great Arteries (TGA)?


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Peter Gruber, MD: Transposition of the great arteries is a complex congenital heart lesion where the two great vessels that come out of the heart-- the aorta, which takes blood from the left ventricle to the rest of the body and the pulmonary artery, which comes off the right ventricle and goes to the lungs-are, essentially, switched.

Sarah Tabbutt, MD: The blood that comes back from the body goes back out to the body again. And the blood that comes back from the lungs goes to the lungs again. And, obviously, that doesn't work.

Thomas Spray, MD: Blood is just going in two separate circulations in a circle, and you don't get any oxygenation to the body. So these children can get into trouble very quickly after birth.

Jack Rychik, MD: In order to understand and to comprehend any of the different forms of the heart disease that exist, it's very important to make sure we understand what the healthy or the normal heart looks like. The normal heart has two sides-- a right side and a left side and four chambers--the top receiving chambers or atria and the lower chambers, which are thick-walled pumping chambers, called ventricles. The red blood cell will come from either the superior vena cava or the inferior vena cava and enter into the right atrium. The blood then flows across the tricuspid valve to the right ventricle. The right ventricle then squeezes and ejects that blood cell into a vessel called the pulmonary artery. Pulmonary artery splits into two vessels each going to the lungs. As that red blood cell makes its way through the lung, it returns through the pulmonary veins to the left atrium. That blood is now oxygenated. It's picked up oxygen then goes across the mitral valve into the left ventricle, which does most of the work, in terms of delivery of blood flow to the body. That blood cell is now ejected into the aorta to some organ or muscle or skin in the human body. Now, there are some significant differences between the heart in the newborn and the heart in the fetus.

Elizabeth Goldmuntz, MD: The heart actually assumes its almost complete anatomy often before a woman would even know she was pregnant.

J. William Gaynor, MD: The heart is actually functioning and pumping blood to the baby throughout most of fetal life.

Jack Rychik, MD: Because the lungs are collapsed in the fetus and it's really the placenta through which much of the oxygenation takes place through the mother, there are various bypass pathways within the fetal heart that direct blood away from the lung. The first is a structure called the foramen ovale. That's a communication between the two top chambers of the heart that allows for blood to go from the right atrium to the left atrium. In fact, because there is very little blood that's returning from the lung, which would normally go to the left side, the majority of blood that fills the left atrium and left ventricle is coming across the foramen ovale from the right side. Because the lungs are collapsed, there's high pressure, high resistance in the lungs as blood is ejected out the right ventricle and enters into the main pulmonary artery, very little goes down into the lungs themselves. The majority goes into a structure called the ductus arteriosus, which is the second point in communication between the pulmonary artery and the descending aorta. The third structure that's important that connects the umbilical vein to the fetal circulation is a site, a junction, called the ductus venosus. That acts as somewhat of a resistor, if you will, in terms of controlling the return of blood from the placenta to the fetal circulation. Birth is a wonderful process and an amazing process, and there's a dramatic change that takes place in what we call the fetal transition-- this transition of the circulation from a fetal life to neonatal life. As soon as the cord is clamped, the ductus venosus ceases to carry blood to the heart, and it begins to constrict within the first few hours or days of life. The very first thing that happens when the fetus is born is it takes its first breath, the lungs expand and so the resistance or pressure in the lungs drop and that promotes blood flow into the lung itself. The ductus arteriosus begins to constrict and is typically fully closed within 24 to 48 hours of life, and blood is now then fully directed into the lung. As the blood returns to the left side of the heart, after traversing the pulmonary circulation and picking up oxygen, pressure in the left atrium rises just a bit and the trapdoor of the foramen ovale, which was open before birth, now begins to close, usually within the first few days of life.

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