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Helen B. Taussig is known as the founder of pediatric cardiology. She became famous through her work with tetralogy of Fallot, a congenital heart defect in which oxygenated and deoxygenated blood enters the circulatory system resulting from a ventricular septal defect (VSD) right beneath the aorta. This condition causes newborns to have a bluish-tint, cyanosis, and have a deficiency of oxygen to their tissues, hypoxemia. She worked with Alfred Blalock and Vivien Thomas at Johns Hopkins Hospital where they experimented with dogs to look at how they would attempt to surgically cure these "blue babies." They eventually figured out how to do just that by the anastomosis of the systemic artery to the pulmonary artery and called this the Blalock-Taussig Shunt.

Tetralogy of Fallot, pulmonary atresia, double outlet right ventricle, transposition of the great arteries, truncus arteriosus, total anomalous pulmonary venous connections, and Ebsteins anomaly are various congenital cyanotic heart diseases. Congenital cyanotic heart diseases is where something is wrong with the heart of a newborn and it is not oxygenating the blood efficiently.

Tetralogy of Fallot

Tetralogy of Fallot

Tetralogy of Fallot is the most common congenital heart disease arising in 1-3 cases per 1,000 births. The cause of this defect is a ventricular septal defect (VSD) and an overriding aorta. These two defects combined causes deoxygenated blood to bypass the lungs and going right back into the circulatory system. The modified Blalock-Taussig shunt is usually used to fix the circulation. This procedure is done by placing a graft between the subclavian artery and the ipsilateral pulmonary artery to restore the correct blood flow.

Pulmonary Atresia

Pulmonary Atresia happens in 7-8 per 100,000 births and is characterized by the aorta branching out of the right ventricle. This causes the deoxygenated blood to bypass the lungs and enter the circulatory system. Surgeries can fix this by redirecting the aorta and fixing the right ventricle and pulmonary artery connection.

Double Outlet Right Ventricle (DORV)

Double outlet right ventricle is when both great arteries, the pulmonary artery and the aorta, are connected to the right ventricle. There is usually a VSD in different particular places depending on the variations of DORV, typically 50% are subaortic and 30%. The surgeries that can be done to fix this defect can vary due to the different physiology and blood flow in the defected heart. One way it can be cured is by a VSD closure and placing conduits to restart the blood flow between the left ventricle and the aorta and between the right ventricle and the pulmonary artery. Another way is systemic-to-pulmonary artery shunt in cases associated with pulmonary stenosis. Also, a balloon atrial septostomy can be done to fix DORV with the Taussig-Bing anomaly.

Transposition of Great Arteries

Dextro-transposition of the Great Arteries

There are two different types of transposition of the great arteries, Dextro-transposition of the great arteries and Levo-transposition of the great arteries, depending on where the chambers and vessels connect. Dextro-transposition happens in about 1 in 4,000 newborns and is when the right ventricle pumps blood into the aorta and deoxygenated blood enters the blood stream. The temporary procedure is to create an atrial septal defect (ASD). A permanent fix is more complicated and involves redirecting the pulmonary return to the right atrium and the systemic return to the left atrium, which is known as the Senning procedure. The Rastelli procedure can also be done by rerouting the left ventricular outflow, dividing the pulmonary trunk, and placing a conduit in between the right ventricle and pulmonary trunk. Levo-transposition happens in about 1 in 13,000 newborns and is characterized by the left ventricle pumping blood into the lungs and the right ventricle pumping the blood into the aorta. This may not produce problems at the beginning, but will eventually due to the different pressures each ventricle uses to pump blood. Switching the left ventricle to be the systemic ventricle and the right ventricle to pump blood into the pulmonary artery can repair levo-transposition.

Persistent Truncus Arteriosus

Persistent truncus arteriosus is when the truncus arteriosus fails to split into the aorta and pulmonary trunk. This occurs in about 1 in 11,000 live births and allows both oxygenated and deoxygenated blood into the body. The repair consists of a VSD closure and the Rastelli procedure.

Ebstein's Anomaly

Ebstein's anomaly is when the right atrium is significantly enlarged and the heart is shaped like a box. This is very rare and happens in less than 1% in congenital heart disease cases. The surgical repair varies depending on the seriousness of the disease.