Congenital Heart Defects

Tetralogy of Fallot is the name given to a combination of four congenital heart defects. Tetralogy means “a group of four,” and Fallot is the name of a French physician, Etienne Fallot, who wrote a book about this condition in 1888. The four defects that make up the tetralogy group are described below:

1. Ventricular Septal Defect (VSD): Sometimes a baby is born with an opening in the wall of tissue called “septum” that divides the heart into the left and right sides. This defect may appear in the wall between the two lower chambers called “ventricles” or between the two upper chambers. As a result of this defect, blood does not circulate normally in the heart. Specifically, not enough oxygenated blood goes through the aorta of the heart to nourish the cells of the body, and the child may be undersized. The heart constantly overworks to make up for this deficiency and may become enlarged.
2. Overriding Aorta: Instead of rising solely from the left ventricle, the aorta straddles both ventricles, rising from a point just over the septal defect. Resulting from this defect, the aorta receives both oxygenated and unoxygenated blood and sends this poorly oxygenated mixture through the body.
3. Pulmonary Stenosis: There is a narrowing of the opening in the pulmonary valve, which obstructs the flow of blood through the pulmonary artery to the lungs.
4. Enlarged Right Ventricle Wall: Due to the strain of pumping blood through the narrowed pulmonary valve, the right ventricle overworks, becomes enlarged, and is not able to function effectively.

The main result of this combination of congenital heart defects is that the blood and the main body tissues do not get enough oxygen. Since blood which is low in oxygen is bluish-red, this gives the baby’s skin a blue tinge, also referred to as a “blue baby”. Treatment of tetralogy of Fallot was initially performed as a closed heart procedure dating back to 1944. These operations did not bring about a complete cure, since they did not eliminate the original malformations but only relieved their effect. Complete treatment of this congenital heart defect did not become possible in certain cases until the late 1950s with the use of an open heart surgical procedure. As more cardiac surgical teams became familiar with this technique, the treatment of this congenital heart defect came into more general use.

With her diagnosis and treatment of tetralogy of Fallot in the late 1950s and early 1960s, Lori Ankerud is one of the very early patients to survive and thrive with this congenital heart defect. Credit must be given to the remarkable care provided to her as an adult patient from world-class cardiologists, Dr. Richard Liberthson and Dr. Doreen Defaria, cardiothoracic surgeon, Dr. Thomas MacGillivray, and the cardiac critical care nurses at the Massachusetts General Hospital.