Shaun White: An Inspiring Story of Accomplishment Despite Congenital Heart Defect
Shaun White, 23 years old and Gold Medal Winner of the Men’s Halfpipe at the 2010 Vancouver Winter Olympics, began his life with a congenital heart defect known as Tetralogy of Fallot (TOF). Before he was one year old, Shaun underwent two surgeries, but know competes with the best of the best in both Olympic and X-Game events.
Tetralogy of Fallot is the most common cause of “blue baby syndrome” or cyanosis, and occurs in approximately 400 per million live births. It occurs slightly more often in males than in females. TOF involves four specific abnormalities in the anatomy of the heart.
The first is pulmonary stenosis, where a narrowing occurs at the right ventricular outflow tract at the pulmonary valve resulting in a reduction of blood flow to the lungs. It is the major cause of the other malformations of the heart, because they disfigure in an attempt to compensate for the decreased blood and oxygen flow. The degree of stenosis varies between individuals with TOF, and is the primary determinant of the condition’s symptoms and severity.
The second abnormality involves the aorta, the largest artery in the body that originates from the left ventricle of the heart and extends down to the abdomen. In TOF the aorta is positioned directly over a ventricular septal defect instead of over the left ventricle, which is called an “overriding aorta”. This results in the aorta receiving some blood from the right ventricle, reducing the amount of oxygen available.
The third abnormality is the ventricular septal defect that the overriding aorta is positioned over. The ventricular septum divides the left and right ventricles of the heart. Defects to this can include a hole or a thickening of the tissue called septal hypertrophy. In some cases, an abnormality can occur in the septum that divides the atrial chambers of the heart, resulting in the “Pentalogy of Fallot”, because a fifth defect has occurred.
The fourth abnormality involves the right ventricle of the heart, which is larger than normal and called “right ventricular hypertrophy”. This causes a reduction of blood flow from the heart into the lungs.
A total surgical repair of the defects is accomplished by an operation known as the Blalock-Thomas-Taussig procedure. Surgery is often carried out in infants one year of age or younger, and over time the mortality risk has decreased to 5%. It is an open-heart surgery that relieves the right ventricular outflow tract stenosis and repairs the ventricular septal defect with a patch or graft. Patients may require a second reconstructive surgery.
Patients undergoing surgical repair of the defects, such as Shaun White, often grow up to have excellent cardiac function with very little exercise intolerance although they still carry some risk for sudden cardiac death or heart failure and need to be monitored regularly by a cardiologist who specializes in congenital heart defects.