I was born on December 31, 1970 in Allentown, PA. They immediately found a murmur, and knew something was wrong. They sent me to St. Christopher's Hospital for Children where I was diagnosed with Tetralogy of Fallot with Pulmonary Atresia. My mother was told that this defect was a very serious and dangerous variation of tetralogy. They sent us home and I started to get sick and turned blue. Seven weeks later I was brought back to the local hospital. I was gray and could not breathe. What happened was the pulmonary aorta ductus that we all have at birth closed like it was supposed to, and I went into congestive heart failure.
My Tetralogy of Fallot with Pulmonary Atresia was described as this: My VSD was about the size of a half dollar, the pulmonary artery was extremely narrow (including the branching arteries) and I was missing my pulmonary valve. So at the age of seven weeks I am in the hospital, gray and unable to breathe. The doctors thought that I was not going to survive long and they tried to make me comfortable. Three days later. The doctors, not believing I was still alive, had me transferred to St Christopher's Hospital for Children in Philadelphia. Surgeons performed one of two shunt surgeries I would receive. My mother said I came back pink. A total change. After that surgery I would get regular check ups and at age 3 they discovered that I only had one functioning kidney. I have had numerous heart caths.
In January of 1977, they decided to try total correction. Unfortunately the previous shunt messed up my right pulmonary branch and they also determined that my heart may not take the pressures that the total correction would produce, so they ended up doing the second shunt. I believe the two I had were the Blalock-Taussig (B-T) and the Waterston shunts.
In October of 1981, and after many more tests and caths (etc.), they decided to perform the total correction. I went in for a 7-1/2 hour surgery and came back 17-1/2 hours later. There was trouble. When they tore down the one shunt that was attached to the aorta, the one suture did not hold. Over a 36- hour period (I think), 50 pints of blood was pumped into my 53 pound body. In total I was opened up three times for that surgery. After or during all that, the ventilator I was on failed. My surgeon hand-bagged me until they got it working again. The surgery they performed was this: They patched the VSD, scraped the right ventricle thinner and bypassed my pulmonary artery with a tube that had a porcine valve in it. I now have a deformed chest because of it.
I was in the hospital 28 days and I quickly became convinced I would never see home again. I was told that later I would need the have the tube replaced as I grew. And that is what happened in 1988.
In September 1988, I was a senior year in high school. The surgery was originally scheduled for August but an emergency occurred at the hospital, and they rescheduled it for September. Knowing about my last surgery, they were prepared. The had to do two cuts because the tube, by way of scar tissue, had fused to my sternum. The surgery went smoothly and better than expected. I was home a week later. They put a homograft in place of the tube and they used part of my pericardium to widen the branching pulmonary arteries. They previously attempted to perform a balloon dilatation without much success.
In 1993, an echo showed that my aortic valve was leaking quite a bit, the branching pulmonary arteries were becoming blocked and that the cardiac output was not what they wanted. They began thinking aortic valve replacement and possibly a transplant.
In September of 1993, I had another cath. In the prep room they explained that they would be putting in a stent to open up the branching pulmonary arteries and then decide about the aortic valve. Well, the cath took a lot less time then normal. They did not need to put in a stent, there was no blockage. They also said that the aortic valve was leaking 4-5 percent and they don't consider replacement unless it is 65 percent or more. The cardiac output was not excellent but fine. The only negative was that the pulmonary valve had calcified and was no longer working and that did not concern them too much. I should be fine as I was for at least 20 years. They sent me to have a checkup at Temple and Temple said they won't need to do anything unless I complain about something.
In January of 2000 I had a stroke and the cardiologist I started seeing recommended for me to go to Temple and be checked out again. My family physician agreed.
I had a echo done July 18th 2001 at Temple Children's hospital. We learned that as kids with my condition or similar with pulmonary valve problems began reaching their 20s and 30s in the 1990's they started to have some major problems. They used to think that they could let the pulmonary valve go and fix the other major problems without too much of a problem with the pulmonary valve and enlarged ventricle. They were doing well and it is part of a low pressure system so it should be fine. Well these kids when reaching their 20s with such enlarged ventricles were getting bad arrhythmias (especially tachycardia) and were dropping over dead. My pulmonary valve was part of a conduit that is a homograft (an aorta and aortic valve from a cadaver used for my pulmonary artery and valve). The conduit itself was fine with no significant obstruction. They found a little narrowing in the right pulmonary branch. But the valve fell apart and I had bad regurgitation into the right ventricle, which they knew about. A normal right ventricle is about 1 cm; in 1993 mine was dilated to about 3 cm. The echo showed the RV at 5 cm. They were very concerned. When the ventricle dilates it damages the muscle and heart muscle does not regenerate. They believed that putting in another conduit with valve in should help. Question is: is my right ventricle too damaged. A stress test was done to determine that. I did not pass the test but I also did not fail it too bad so they believed surgery should help quite a bit. They determined to see what another echo showed in 6 months
Good news is I didn't had too bad of arrhythmia yet. And since I was doing so well I should tolerate any surgery very well. The downside is I probably have a lot of scarring inside and that is prone to bleeding so they would have to be very careful.
After 6 months the RV enlarged to 6 cm. It was time for surgery. It was planned for May 2, 2002 at Children's Hospital. I had the surgery but ran into some bleeding and rhythm issues. I had a stress test the next January and I got up to about 134 bpm when the heart jumped to over 200 bpm and stayed there for about 2 minutes. I had no symptoms and the heart threw a PVC and the rate dropped. Since them they decided to implant an ICD which was done July of 2004. My right ventricle is non-functional and it does not seem that it will come back.
I am now going on with a bovine valve (I suffer cow jokes...) and an ICD (suffering from my wife asking the doctor for the remote control) and I'm stable for now.
