My Heart History

When I was born I had several things wrong with my heart. I had a Patent Ductus Arteriosis that needed repaired at the age of 10 months. I continue to have aortic stenosis that has become more severe as I've aged. I also have a malformed aortic valve that is a variation of a bicuspid valve. (Your aortic valve is supposed to be a tricuspid valve having three leaflets that open and close to let blood flow through in one direction. A true bicuspid valve only has two leaflets. Mine has three leaflets, but two of them are fused together.) Because of this malformation, I have a moderate leakage back through the valve which is known as regurgitation. I had two cardiac caths done growing up, one at the age of 5 and one at the age of 15. I have a very loud murmur and have been monitored by cardiologists my whole life. Well, not exactly, as I failed to concern myself with cardiac care once I graduated from college. I had no idea that surgery was guaranteed someday, I probably would have been a little more concerned had I known. I had a fairly frank conversation with my pediatric cardiologist when I was about 16 years old. Asking all the pertinent questions, such as can I have a child someday, and are my conditions hereditary. He said I could have children, my conditions were not hereditary and the only thing I really needed to worry about was the build up of plaque due to cholesterol. (Concern for high cholesterol was new at that time, he had me scared to eat potatoes after that conversation - I remember him specifically naming potatoes and lots of butter as foods to avoid.) No mention of future surgery - so I went on to get married and have a baby, without any cardiac care for close to a decade. After I gave birth, the strain of a natural child birth (prolonged by back labor) jumpstarted the inevitable increase in a change of pressure within my heart, also referred to as the gradient. I wasn't feeling so well, and wasn't handling physical activities as well, so I decided to see a cardiologist. After a series of echos, she very bluntly informed me that my gradient has started it's increasing path and once this increase begins, it doesn't retreat. I would need surgery one day, but not until I am very old. Old or not, the prospect of surgery was terrifying. I tried to put it out of my head, as very old would be a long ways a way. I faithfully returned, with more complaints each year and cared less and less for this cardiologist with each visit. One visit, she was going to be out of the office, so I had to see one of her partners, Dr. Gurmeet (Gary) Singh. He was very concerned with my care and I've been with him ever since. Several years passed and I wanted to have another baby. I humored mom by asking Dr. Singh to okay me for childbirth. I was very surprised when he said no. He wanted me to have a
Trans-esophogeal Echo (TEE) before he would okay me for a pregnancy. During a TEE, you are asleep and they put a scope down your esophugus to do the echo internally. Not a pleasant experience, but worse was when I started to awake and heard all the commotion in the operating room. People were crowding around to see my "aortic shelf" on the monitor. No one would explain to me what it was at the time, but during my post office visit with Dr. Singh, he explained to Tony and I that I have a subaortic membrane that sits just below my aortic valve, which creates even more difficulty for my heart to pump blood through the valve. He explained that this is a very rare condition and no one in Delaware would be qualified to handle what to do about it. My feeling was that there was nothing to do about it. It had been there my whole life, it can just stay there. Surgery would wait until I'm very old, that had already been decided. I just wanted to know if I could have a baby. I was sent to Deborah in New Jersey where I was told there was something there, she doesn't know what it is, but don't worry about it, have a baby. (That was very strange to process) I went to University of PA and was told that I needed to deal with this immediately, but my insurance refused anything more than an office visit. Next, I was sent to Hopkins and was told that I didn't have a membrane, but I needed my valve replaced and he could do the ROSS procedure in three weeks, but first see the pediatric cardiologist for a second opinion. (At this point Tony and I were quite afraid, I just wanted to know if I could have a baby and now I was having to process the very scary idea of the ROSS procedure.) The pediatric cardiologist confirmed that there was a membrane but was 50/50 on doing the surgery at that point so he sent me to the head of their cardiology dept. We traveled back to Hopkins for a third visit with the department head, who told me there was no membrane and basically what I was feeling was not cardiac related. I was so mad, I can't remember if he said to have a baby or not, but by then there was no way Tony was agreeing to take that risk. Dr. Singh knew I was not seeing anymore doctors, so he did a cardiac cath to determine if the gradient (pressure change) occurred before the valve or across the valve. It occurred before the vavle, which confirmed the membrane was there. (I don't know that I was 100% conviced, but he was.) We decided to leave well enough alone and watch for symptoms. Again, I'm thinking surgery would be when I'm very old. God, apparently, has other plans. The past few years I've felt more and more sluggish with other symptoms creeping in. Last August, my gradient had jumped into the 60's so Dr. Singh wanted me to do another TEE. (Previously my gradient had been in the mid 30's and eventually the 40's. The jump to 60 happened in a very short period of time.) I agreed to do the TEE in November and the TEE showed what may be a second membrane. It also confirmed the gradient was in the 60's and I was told that I needed to do the surgery. I agreed to see the specialists at University of PA in January and have been on a rollercoaster ride ever since. (Between deciding on what type of valve to fighting with the insurance company, to facing my own mortality - it's been quite a journey already.) A wise man told me that I may look back on this year as the best year of my life - I already see the gifts in this experience, frightening as it is. I know the support of my family is a blessing, my faith is stronger, and my outlook on what truely matters in life is far more focused. I am better able to appreciate all the little moments with Tony and Ashley and I doubt I'll ever take those moments for granted again. Thank you Mark, for your wise words.