4.5 Ascending Aorta - PCP recommends general cardiology

If your child has Ehlers-Danlos Syndrome, your PCP is off base on saying there is no connection.
Most primary care physicians and many general cardiologists are clueless about thoracic aortic aneurysms.

Can you self refer to another cardiologist and or cardio thoracic surgeon?

Connective tissue disorders definitely need to be ruled out.
Testing can be expensive and may or may not be covered by your insurance.

5.0-5.5 cm aneurysms are generally considered for surgery, but with connective tissue disorders it could be as low as 4.5 , I believe …

I was originally seeing a general cardiologist when diagnosed with an AAA, but through my own perseverance, I found a cardiologist that specializes in genetic heart issues (my 4.0cm AAA is most likely hereditary since my brother had an AAA and there are lots of deaths at young ages due to heart ailments in my father's family). The info and treatment has been like night and day between the general cardiologist and the genetic cardiologist. A subsequent scan found a dilation on my celiac artery which sent my anxiety into a tailspin worrying about other aneurysms and started a conversation with the genetic cardiologist about a possible connective tissue disorder. I have since had a brain scan and full abdominal scan to rule out other aneurysms. Thankfully I have insurance that does not require a referral to see a specialist so I was able to see the genetic cardiologist without a referral.

I have not seen a surgeon since my genetic cardiologist seems to have everything under control. He said he will want me to see a surgeon if/when my AAA is 4.5cm due to my family history and possible connective tissue disorder. My theory for not seeing a surgeon now is that I may not need surgery for may years, and if I see a surgeon now, there is no guarantee that the same surgeon will be practicing at an office in my insurance plan when I'm in need of surgery. Of course I will definitely continue seeing the genetic cardiologist on a regular basis.

In my humble, unprofessional opinion, you need to see someone ASAP who is highly knowledgable in genetic heart issues and connective tissue disorders.

I agree strongly with @rlhix and @eawarl. Ehlers-Danlos syndrome is inheritable and it clearly makes a big difference in the management of an aortic aneurysm. (I'm not sure why your PCP would say that. You could ask him/her to explain.)

Mayo has links to request an appointment. Cleveland Clinic does as well I think. With a little googling, you should be able to find an aortic center within the most acceptable travel distance and they will most likely have a similar link. What is there to lose by giving this a try? Just make sure your insurance covers it before you go.

I would find it stressful to "go around" my PCP. But it's not worth dying over. Thankfully, mine is great and I haven't had to yet. I did ask for a referral that "superseded" one of hers, but she was glad to do it. Yours may be too.

Good luck! Keep us posted.

Maybe tell us what part of the United States (or elsewhere) you live. I am in Minneapolis and the University of Minnesota as well as Mayo have excellent heart programs. I had my successful surgery done at UCSD La Jolla, California. Respectfully, your primary care doctor is wrong. Keep them for your annual checkups and COVID-19 shots. Get in with a THORASIC SURGEON who specializes in Aortic Dissection repair. Not a cardiologist. You need the person who will ultimately be cracking your chest open, if necessary, to repair your aorta. Trust me, you do not want it to dissect on its own. That's exactly what happened to me in 2015 and I would not wish it on anyone. I would guesstimate 98% of all doctors, nurses, and first responders are unfamiliar with exactly how dangerous an aortic dissection is. You need to get in front of the 2% who do. I watched a Cleveland Clinic aortic dissection detection seminar on Youtube with hundreds or doctors and nurses in attendance. Only two of them knew what a dissection looked like on a CT scan. Peace. Watch this: https://youtube.com/shorts/u-f3AmX1tv4?si=694rH-Bn6KMfflMP

@moonboy I appreciate your reply. I read your other posts and commend before meeting with my NP but she wasn’t convinced that I would ever need surgery. Perhaps I won’t, but I’d rather a surgeon tell me that and be the one making the call. See below.

My PCP is a NP Northwestern Hospital in Chicago. I don’t think I’ll have to travel to find a surgeon, but I do need a referral to get an appointment with one.

She recommends:
Mohamed M.H. Al-Kazaz, MD | Northwestern Medicine. He is not a surgeon but seems to have aortic aneurysm as a specialty.

His first available is in January, however today he replied to my NP to tell her to order:
-CTA aorta assuming renal function is still good
-ECHO 2D Doppler to look for bicuspid aortic valve, aortic regurgitation ..etc.
-12 Lead ECG
-Consult genetics (cardiology). Long wait time but place order now and she can schedule follow up and once we see her then we can see if we can expedite it based on her physical exam for CTD.

Also consider:
-BP diary and strict control < 130/80 if not 120/80. We usually like BB and/or ARB not just for BP but also to slow down progression of disease based on data from marfan's patients but we use it for all
-restrict weight lifting < 50 IBS to avoid increasing risk of dissection
-avoid levaquin or similar drugs.

——

So, it seems like the ball is rolling now. I also have a phone appointment next week with a cardiac nurse in the Northwestern Cardiovascular Clinic. They have a program called FAST designed to get info and specific recommendations for newly diagnosed patients. I scheduled it before I heard back from my pcp. Hopefully it won’t take months to get these tests done.

My next question may seem basic, but whenever I try to take my BP at home I get errors. It gets errors when someone else tries to do it for me, on either arm too. The machine works and gets readings for other people’s arms but for not for mine. And, it’s also excruciating every time the cuff inflates and even afterwards. Does anyone have a recommendation for a good BP machine?

Ok- that’s enough for now. I’m outside in the heat and freaking out because I do not have enough into yet about my condition. Going home to the AC and maybe stopping at Walgreens to see if I can get my BP.

I’m in Minneapolis sitting on the heat myself right now. Here’s what I know about the blood pressure thing, the only one I’ve ever found that I thought was useful was the Omron wrist cuff. You might wanna order that off Amazon and try it. I don’t really get too riled up about my blood pressure. I’m taking amlodipine, atorvastatin, and carvedilol in sufficient quantities to lower my blood pressure. At my last check up last week it was 118/74 so I’m pretty happy about that. What I’ve learned is that caffeine does not raise my blood pressure but sugar does. It’s odd to think that sugar raises your blood pressure, but it actually does. So I would definitely not be doing any sort of planking type exercises or anything involving deadlifts or anything more than 20 or 30 pounds. The goal is just to bring down the temperature in the room as it were. I Think that if you get in to see a thoracic surgeon you’re gonna have a really good clear picture of where you’re at physically and what their plan is for you.

In the meantime, you’re gonna be fine. It’s all good. I have survived for the last 10 years with an artificial aorta and I promise you this that you’re doing the right thing by staying on top of it. If there’s one piece of advice, I could give anyone who’s facing aortic issues. It’s this: do not take advice from anyone who isn’t a thoracic surgeon who repairs aortas or a person who has survived an aortic dissection. In a perfect world, you would have a thoracic surgeon who has survived his or her own aortic dissection! I really mean it.

There are a lot of medical people who have opinions about things that they really know nothing about because they’re expected to be smart and have opinions. i’m a lawyer, and I understand the phenomena of expecting that a lawyer will always have the right answer. Obviously, we don’t and neither do the doctors. You need to get in to see a thoracic aorta surgical specialist. They will take good care of you. Peace.

I think the only way to a surgeon is through a preventative cardiologist. He or she will get you a referral for genetic testing. Mine did, Patricia Vassallo, at Northwestern. I am seeing Doug Johnston there for surgery now for bypass and aortic repair. I am symptom free, so it has been a surreal journey. My dilations are generally not large enough for surgery (4.4 and 4.7), but I also have multiple blockages in my arteries, so they will fix the aorta at same time as bypass.

My journey started with Calcium Score test too. That’s when the dilations were found. I also went to Cleveland Clinic, and the first screen there is with a cardiologist. Same recommendation for surgery. So far, happy with Northwestern.

Do your research first to find the best cardiovascular surgeon in the hospital you want to be treated. Then contact Medicare to find you a PCP to evaluate your health history and referral to the specialist surgeon.
Good luck.
Tainan.

I agree strongly with @moonboy. I have a 4.3 cm descending thoracic aortic dilation with “pseudoaneurysmal outpouchings” and have thankfully not had a dissection. I was also diagnosed 12 years ago with a “non specific connective tissue disorder.” The cardiologist I thought I trusted back then told me I didn’t need to be scanned any more and that there was nothing else that could be done, so I should just go live my very active life and take my statins and if it ruptured, they would take care of it then! I was at 2.9 cm then and believed her. Last year before I started on a permanent RV journey across the US, I insisted my then cardiologist order a scan just for the heck of it. I had met my OOP deductible for the year so it didn’t cost me anything. Then she flipped out when she saw the results and sent me to a vascular surgeon, convinced I needed surgery immediately! Vascular surgeon in town thankfully has a lot of experience repairing aneurysms and said nope, no surgery and go live your life doing whatever you want but come back every 6 months for a CT. Second one, it had grown another couple of mms and he’s like yeahhh, you’ll probably need surgery one day. I’m like yeahhhh, I need to get myself to an aortic disease center.

All this is to say, you have to be extremely proactive and take charge with all the knowledge you can acquire until you can get into see an experienced vascular surgeon at an aortic disease center clinic. There are many now around the US as aortic disease research is slowing advancing (especially as boomers age, you see more cases and so more research and clinics popping up). I’m mainly in Florida, so I sent a msg from the web page form to both Mayo Clinic and University of Florida-Gainesville that also has a highly rated program. I see Dr. Tomas Martin there next week. Took a couple months to get the appointment but I’ve already had the genetic counseling appointment through the UF aortic disease clinic. Dr. Martin’s clinical coordinator took care of getting all my records which was awesome. And reiterating, most cardiologists have ZERO clue about aortic aneurysms. So you have to find one with experience and interest in aneurysms and vascular disease.