My Journey with FH and Heart Disease

Journey to Vascular Health: Adding a Specialist to FH Team

Blog Post from My Journey with Familial Hypercholesterolemia and Heart Disease
  You will learn fast that when it comes to FH you very often don’t take just one medication. You take several. You don’t follow up with just one test. You follow up with multiple tests (blood tests, imaging, etc). Very often, you don’t see one specialist, but you see several, as a routine. This is a story about all that. Following up on my January appointment with my cardiologist (, I got an MRI of my abdominal aorta in February. The list of findings was long, but the most important ones are the following:
  1. There is a significant stenosis at the origin of the celiac trunk (>75%).
  2. There is moderate proximal stenosis in the superior mesenteric (~50-75%)
  3. The right renal artery has an acute take off. There is moderate stenosis (eccentric in shape) at the ostium of the right renal artery (~50%).
These are the most important concerning areas that this latest MRI revealed. I will also have to add that for the first time in … I am not sure how long of a time, the MRI tech chased me down the hallway after the test was over and after the nurse took out my IV to ask me about my history. He, of course, did not say anything about the findings, as they never do, but he had a cheat-sheet with hand-written notes in his hand and he wanted to understand my history, my background, and why I had such an involved surgery four years ago ( I wondered when he did that what he found or whether what he found was unexpectedly worse than what he was hoping for… Maybe this is why no techs ever talk with you when you have these tests – because if they do, you start wondering and worrying. I wondered, but not worried. With my history, you take pretty much each day as it comes and try not to expect too much. A couple of days later, I got an email from my cardiologist saying something to the effect that “it all looks good. Abdominal aorta unchanged from the ultrasound we did in December.” I pulled my December results and the findings then were quite different than what this MRI showed. Then, they noted the following: Mild to moderate calcified plaque was identified in the proximal to distal segment of aorta. Elevated velocities were recorded in proximal to distal segment of aorta, suggesting a greater than 50% stenosis by velocity criteria. The bilateral common iliac arteries were visualized (…) with mild to moderate calcified plaque identified. Elevated velocities with biphasic waveforms were recorded in proximal segment of common iliac arteries bilaterally, suggesting a greater than 50% stenosis by velocity criteria. (…) Elevated velocities were recorded in the proximal segment of celiac axis artery and superior mesenteric artery, suggesting a greater than 50% stenosis by velocity criteria. Decreased diameters in caliber were identified throughout. As you can see, the findings were quite different. So, I questioned him. He insisted that the findings are “the same, but the tests are different, so we can’t compare apples-to-apples: one was an ultrasound, one was an MRI, so the measurements are different but the diagnoses is the same: I have disease, but there is nothing they want to do now because it’s not near 90%, so I need to just wait it out.” (pretty much an actual quote from the cardiologist). I did not feel comfortable about his answer. I do like the work he is doing for my heart, but I felt like he was taking my aorta findings quite lightly. I pulled out my operative report and started to highlight all the references the surgeon had made to my “porcelainized aorta” and the “severe stenoses” he had found in multiple arteries. The aortic valve was so calcified only one leaflet (out of three) was functional. And I wanted to sit down with a vascular specialist to understand exactly what kind of a risk my arteries are facing. From my annual heart echo, I have a pretty good understanding of my heart, I think, but I feel like I need a “watch” plan for my arteries just the same, in addition to my heart. When I had my surgeries, the three surgeons that were in the OR all told me the state of my arteries was one of the worst they had ever seen and my own surgeon repeatedly told me that I need to monitor them closely, not only in my heart but everywhere else in my body. So, I pushed for more answers. I asked the cardiologist for a referral to a vascular specialist and apologized if he thought I didn’t trust him – I do, but I need someone more specialized in arteries the way he is in the heart. He didn’t take it the wrong way (I hope) and he made an appointment himself with one of the interventional cardiologists and vascular specialists in the same clinic. I met with this new doctor last week and it was quite a different conversation than I had with the cardiologist. He does manage hearts, but his specialty is all “the other” arteries, outside of the coronary ones. He agrees that I should be equally concerned about my arteries as I am with my heart. He explained that the difference between the ultrasound and the MRI is that the tests are indeed different and the level of detail is higher on the MRI. He would like to monitor me, and get on a yearly monitoring plan, probably a CT – Angiogram scan every year to all the arteries between my jaw and my knees. Although he agreed that the most accurate test would be an MRI (and with the least amount of radiation), he would need to order three MRIs to capture the length of the arteries he wants monitored, whereas with a CT-A, he can order one test. The MRI, although the most accurate, would triple the cost. The main area of concern is my abdominal aorta and especially my celiac trunk, but also the mesenteric and renal arteries, as well as the subclavian arteries which come up as stenotic in the carotid ultrasounds. Now, I add one more test to my yearly plan: in addition to a heart echo, we will add a CT-Angiogram scan which will probably also replace the yearly carotid ultrasound that I normally do. The vascular specialist will work together with the cardiologist to follow the development of my disease closely, both for my heart and for my other areas of my arteries. I’ll have to say, I feel better about having a plan. The cardiologist was maybe going to repeat the tests, but the thing with him is: he keeps doing a different test every time – an ultrasound, an MRI, a CT scan. I felt like we need some consistency, to build a baseline, and to see a clear progression from where we started, over time. This is what I did for the heart and this is how I knew when things were bad enough in the heart that I needed to have surgery. The vascular specialist agrees. He also “promised” me that I will probably have more surgeries in my lifetime to correct some of these areas as the disease in them looks pretty advanced for a person my age. So, watching this closely is that much more important, so we know when we need to act, hopefully before some major event should happen. I guess, the moral of this story is like always: if you have doubts, if you need more answers, ask, explore, push and get them. There is no shame and no guilt in wanting to be in the know about your own body. It is your life, after all! Happy health, you all! And I wish you all answered questions, and answered prayers!   This blog is reprinted with permission from A. Wilson. To view original post visit: Blog Post by A.W. FH Advocate for Awareness - Alina W. About this Blog In this blog I will follow my everyday journey of living with familial hypercholesterolemia (or FH). I am sharing my own experience with this inherited disorder, and how I manage it daily – from what literature I read on the topic and what my doctors say to how I live my life (what I eat, what medicine I take, how I exercise, etc). This is solely a personal account that might or might not offer some insight on what to expect when diagnosed with this condition. This blog does not offer advice, in any way, to anyone suffering from this disease.  

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