What are they fixing now & why?

I'm getting a new pulmonary valve. Why? Because the one I have is tired and needs a tune-up. My pulmonary valve is not closing properly, and had been continuing to get worse as I get older. It is very common for adults with Tetralogy of Fallot to get a new pulmonary valve.

As this valve has gotten weaker, it has been allowing more blood to flow back through it. This is what causes my heart murmur. This backflow causes my heart to have to work harder to get blood thru my weak valve. The continued backflow has been causing my right ventricle to inflate, kind of like a balloon, from all the extra blood coming back thru.

Why operate now? Because if the pulmonary valve is replaced now, chances are good that my right ventricle will "shrink" back down to a normal size. If we wait any longer, and allow the ventricle to get larger, chances are it will not shrink, and will stay inflated regardless of surgical intervention.

I'll be getting a bovine (cow) heart valve. (no vegetarian jokes, please). It's really kind of amazing. They grow a new valve around a mechanical base with tissue from a cow's heart, and is expected to last 10-15 years.

The man with the plan

Okay, the MEN with the plan.

My cardiologist is Dr. Gary Webb. He specializes in Adults with Congenital Heart Defects, and heads a ACHD clinic at UPENN. Lucky me! For more info on Dr. Webb and his clinic check out the link on my link list.

My surgeon is Dr. Joseph Woo. He is the Director of the minimally invasive and robotic cardiac surgery program. For more info on Dr. Woo check out my link in the link list.

Where and when is the surgery?

We have scheduled surgery for Thursday, December 13th. It is being done at the Hospital of the University of Pennsylvania in Philadelphia. Want more hospital information? Check out the link on my link list.

What has already been repaired?

Most of you know, I had open-heart surgery at the age of 2 (1980) at Children's Hospital in Detroit. At that point, they did a "complete repair" which involved:

1. Closing the ventricular septal defect with a patch.
2. Removing some thickened muscle below the pulmonary valve.
3. Repairing or removing the pulmonary valve.
4. Enlarging the peripheral pulmonary arteries that go to both lungs.

What is Tetralogy of Fallot?

Primary four malformations

As classically described, tetralogy of Fallot involves four heart malformations which present together:

1. A ventricular septal defect (VSD): a hole between the two bottom chambers (ventricles) of the heart. The defect is centered around the 'outlet septum', the most superior aspect of the septum, and in the majority of cases is single and large. In some cases septal hypertrophy can narrow the margins of the defect.
2. Pulmonic stenosis: Right ventricular outflow tract obstruction, a narrowing at (valvular stenosis) or just below (infundibular stenosis) the pulmonary valve. The stenosis is mostly the result of hypertrophy of the septoparietal trabeculae, however the deviated outlet septum is believed to play a role. The stenosis is the major cause of the malformations, with the other associated malformations acting as compensatory mechanisms to the pulmonic stenosis. The degree of stenosis varies between individuals with TOF, and is the primary determinant of symptoms and severity. This malformation is infrequently described as sub-pulmonary stenosis or subpulmonary obstruction.
3. Overriding aorta: defined as when the aortic valve is not restricted to the left ventricle, thus having biventricular connections. The aortic root can be moved anteriorly or override the septal defect, but it is still to the right of the root of the pulmonary artery. The degree of override is quite variable, being between 5-95% of the valve being connected to the right ventricle.
4. Right ventricular hypertrophy: The right ventricle is more muscular than normal, causing a characteristic coeur-en-sabot (boot-shaped) appearance as seen by chest X-ray. Due to the misarrangement of the external ventricular septum, the right ventricular wall increase in size to deal with the increased obstruction to the right outflow tract. This feature is now generally agreed to be a secondary anomaly, as the level of hypertrophy generally increases with age.