Congenital Heart Defects

TETRALOGY OF FALLOT 

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. 

CONGENITAL HEART DEFECTS

Tetralogy of Fallot, a congenital heart defect, is not just a birth defect, but a life-long condition requiring specialized cardiac care.  Congenital heart defects are the number one birth defect in the United States with 1 in every 110 infants born with one or more congenital heart defects.  At least 25% of these infants will require heart surgery or medical interventions just to survive.  Most people are unaware that there are more than 2 million Americans living with congenital heart defects with more than half of these people being over the age of eighteen.  Although medical treatment options for this population have advanced over the years, congenital heart defects remains a leading cause of death for children.  Awareness of knowledge about congenital heart defects is vital to making a better life for children and adults living with congenital heart defects.  Through early detection and proper diagnosis, improved treatment options, and advanced medical research, these children and adults will have a far better chance at living longer, healthier lives.  Heartfelt Dreams Foundation believes the published research data indicating that 1 out of every 4 deaths that occur following surgery for the most severe heart defects could be prevented by having the operation performed at hospitals where surgical teams do the greatest numbers of procedures.

SPECIALIZED CARE 

Heartfelt Dreams Foundation is a non-profit charitable corporation dedicated to providing counseling, emotional, and financial support to the families of congenital heart defect patients and the medical professionals who care for these patients. Massachusetts General Hospital (https://www.massgeneral.org/heartcenter/services/treatmentprograms.aspx?id=1003) in Boston is one of the top US hospitals for the diagnosis, treatment, and care of children and adult congenital heart defect patients.  Other leading US hospitals providing specialized care for congenital heart defect patients are shown here: