Another question about CTEPH

[blizzard2014]

Hey, about seven years ago, I had a massive saddle pulmonary embolism. Apparently it did a lot of damage to my lungs. I have recently been diagnosed with a moderately enlarged right heart. My ejection fraction is normal, and my PAP is 20.1. The doc said my PAP is completely normal. No Pulmonary Hypertension. My PCP who is also a lung doctor said that in the absence of an enlarged left heart, it has to be pulmonary hypertension. He told me that on some days the heart can be pumping good and the PAP can be good and not picked up on a test. He also said that since being on meds for systemic high blood pressure, it can sometimes help heart function and PAP. I am on Lisinopril and Clonidine for high BP. I have been checked for chronic blood clots with a VQ scan and it came back completely normal. Chronic blood clots have been ruled out. But we still don't know what's causing the enlarged heart 7 years after the blood clots in my lungs have been cleared oud. I'm just wondering if you can have CTEPH and it not show up on an echo of the right side of the heart. Any advice would be greatly appreciated.

[Katinka46]

Good morning Blizzard

I am very interested in your story. In some ways I have the reverse of your problem. I have chronic thromboembolic disease (CTED) but without pulmonary hypertension.

First question: you talk about your PE causing a lot of damage to your lungs. Do you know what that damage is? Damage caused by PEs, I would have thought, would have been CTED or CTEPH. So what is the nature of that damage?

Second query: you are right that a normal V/Q scan rules out CTEPH. When was that V/Q scan done? CTEPH can take a while to develop. So if it was done very soon after the acute PEs then maybe another one to check?

Third comment: I am intrigued by your PCP (Stands for ?) saying that PAP can change from day to day. Although I had been told that I do not have PH I am convinced I do and that it rises during the course of the day and on exercise. I have had seven echos that show mild PH but on right heart catheterisation (RHC) the pressures are normal. Have you had RHC? RHC is said to be the gold standard for detection of PH but when I was at the Royal Brompton Hospital in London, a hospital with a world-wide reputation for lung and heart disease, the consultant I saw said that they have patients with PH who had normal RHC. So I think a bit of healthy scepticism is needed. Scans can be very difficult to interpret, mistakes can be made, things can change from day to day, and patients must be listened to.

Ah... I think I have worked out PCP, Primary Care Physician? And so you are American?

All the best and I would like to know more.

Kate

[blizzard2014 in reply to Katinka46]

Hello, Katinka. I had my VQ scan about 5 years after my PE's. It was normal. The damage in my lungs was not documented because I did not have health insurance when I went into the hospital. They did not perform an echo when I had my clots. I guess since they didn't see a dilated heart on my CT scan and my vitals were normal, they ruled out acute pulmonary hypertension. That is how most PE patients die, from pulmonary hypertension that causes right sided heart failure and cardiogenic shock. My clots were extensive going into every lobe of each lung, both upper and lower. The blood doc was going to put me on blood thinners for life merely based on how extensive my clots were, that was before they discovered two blood clotting disorders. I just got sub-par treatment because of not having medical insurance; but since Obamacare, I am getting access to more and better specialists.

I have read that you can have pulmonary hypertension that only shows up when you stand up and exercise. I think this is what I might have; as I already have a weird condition where my blood pressure jumps to stroke levels upon standing, with high heart rate, then back to normal after sitting. It is called POTS and Orthostatic Hypertension. I have no official diagnosis, not unless I can be put on a tilt table test, but the meds they give me to treat the spikes are working. I know tests can fail you. I just wonder if how they are done and on good days if they miss stuff. For years I have had high blood pressure and all is normal when sitting. They only caught it a few times when I had just barely sat down and my high standing BP had not leveled out yet. Then I started testing standing up and was going all the way to 190/130 at times, with a heart rate of 150 BPM. I get tired a lot and muscles hurt everywhere with constant headaches. My oxygen levels drop to 90 sometimes when I am walking, but are usually bouncing back and forth between 94 and 95 when sitting down and resting. There is something going on, just hard to pin point.

[Offcut]

I am high PH and had a VQ which for some reason did not light up my left lung hardly at all. They said I had CETPH but later were not sure as my MRI did not show what they expected? The RHC did show I have right heart enlargement and high IPAH but they do not know why? I am on no O2 at all which doing anything above mediocre hard work?

Be wEll