Pulmonary Arterial Hypertension

I will celebrate or cry with you @marybird .

When I first started reading about this condition I, too, was very depressed and scared in particular since the cardiologist/EP I was seeing for AFib was totally unconcerned about it. I had to self refer to a pulmonologist for follow up on the echo. He assured me I wasn’t “on my death bed” which I feared. He sent me to pulmonary rehab saying I needed some exercise (so true, I’d become afraid to do much of anything) and that has helped, just need to keep it (40 min on treadmill and NuStep)up on my own now. My new cardiologist is the one who agreed I could try sildenafil which I just started. I used GoodRX ($17 for one month supply) since my insurance won’t cover it without a right heart cath for diagnosis. I, too, wonder if this is a cure ( but I read there’s no cure) in that it is supposed to ease/lower the pressure, right? Or can it stop the progression?

Do you think when you go off amioderone your pressure will go down? Since I took that too I’ve wondered if it would. I asked dr if he thought amioderone caused it and he said no but of course he would since he prescribed it! I started on that when fleccainide quite working for me, took it for 7 months till my ablation last February.

Furosemide is a diuretic- I wonder why you were given that for PH? Were you retaining fluids badly? The sildenafil is supposed to dialate the pulmonary arteries which would be what we want to happen I think.

I haven’t been on Amioderone for a few years. Glad it is history and yes, I think it contributed to my PH. Amioderone can cause lung problems.
It can drive you nuts - as you say - trying to stay on top of everything.

Oh thanks! I very much appreciate that.

Well, when my cardiologist got the results of my echo and saw the elevated pulmonary artery pressure ( 68 mm/Hg, their upper range of normal is 36 mm/Hg), he said the first thing to do was to try the furosemide for several months. I've had scans, x-rays over the years and there's no evidence of any lung disease and I'm thinking the cardiologist may be thinking it could be due to possible left heart disease ( mild to middlin at this point). I have had some ankle and leg edema, some unexplained bloating in the middle which has, I think, been helped by the furosemide. I saw my PCP after that couple months was up, had this discussion with her and she declared, in no uncertain terms, that I needed to continue the furosemide and she renewed the prescription. I saw the cardiologist several months later, and he agreed with continuing the furosemide and he ordered the followup echo and said we could go from there depending on the results. I guess this sounds kind of cavalier on the part of the doctors, but their decisions have been based on the fact that I'm for the most part, asymptomatic, am not hypoxic and am having no troubles breathing. From what I'm reading it sounds as though at least some of the medications that dilate pulmonary arteries are given to relieve the symptoms PH patients suffer?

I was also reading more about PAH and PH, and see that diuretics can be used to help with the edema, lowering fluid levels and making the work on the heart and lungs easier in people with PAH or PH. I know they are hoping that my upcoming echo will show lower pulmonary artery pressures as a result of the diuretic. If the pressures are still elevated, we will go from there.

Thing is, from what I am reading they don't recommend the medications that dilate the pulmonary arteries until they have 1) confirmed a diagnosis and determined the type of PH a person may have, and 2) these medications are not recommended for people with left-sided heart disease. As I see it, we don't know where I am, or indeed if I really have PAH, and I'm not feeling bad, so it's a bit soon for the meds commonly used to treat this condition. But the furosemide has helped some of what I was experiencing. That's my take on it, anyway.

Thanks again for sharing your experience. Keep us/me posted regarding the result of your echo. It would be wonderful if all a person had to do to "cure" PH was to take a diuretic as you've done! No one has suggested that to me "yet" anyway. My echo pressures were 46 and 41 and they told me that was very mild and that they didn't get "concerned" until it was 50s, 60s, 70s. I AM concerned however! I wonder why they didn't want you to have a right heart Cath procedure to confirm your diagnosis with your echo pressure being 68?

Well, I can say everyone was very surprised at my echo pressure being that high, I know I certainly didn't expect that. I honestly don't know why they didn't follow that result up with at least a repeat echo sooner than the one I have upcoming on June 12th, or possibly a right sided cath, but honestly I was happy they didn't and I didn't have to face these things ( especially the cath) and could just wait and see if the furosemide helped. I'm sure I'd have felt differently if I were symptomatic, but honestly it doesn't seem as though things have progressed symptomwise in the last year or so. I didn't want to rock the boat, so to speak, by insisting they do further testing at that point. So I laid low and took the meds!

We'll see what the upcoming echo brings, I'm sure there will be more testing in my future should the pressure continue to be as high as it was. As I'd mentioned in an earlier post, I've had several chest CT scans ( for cancer surveillance) that all noted a significantly enlarged pulmonary artery base ( including measurements) that suggested pulmoInary artery hypertension, so it would seem that something is afoot. I'd say that if the pressure is significantly lower ( thanks to the furosemide, or lower salt diet, or my dogging through exercises, whatever it is), I probably never had PAH/PH at all, but I don't know what would have caused the high pressure and enlargement.

I don't know if this is the most realistic way to look at things, but I've had a number of health scare/issues in the last few years ( including colon cancer), and other serious health issues in my close family members. I've always been a "half-glass full" type gal and have found the best way to deal with these issues was to look on the more positive side of things. I've been lucky in that we're all doing well with our issues and things have, at least so far, turned out as well, or better, sometimes, than could be expected. At this point, as I told my oncologist at one point, that I "was not going to worry about anything until someone who knows what they are talking about tells me it's time to worry". He agreed with me that this was a good way to look at things, though I know it's easier said than done for many people.

So, we'll see about my "furosemide cure" for PAH! Just kidding, but who knows?

It's hard to know what to do - the EP cardiologist I saw for Afib ordered the first echo last November which showed the mild PAH (scared me death, first I knew of this condition) and then never said a word about it. I asked the nurse at that time shouldn't this be addressed and she said "we don't deal with that issue" you'll need to see a pulmonologist and sent me out the door.

I self-referred to pulmonary and it has been downhill from there so to speak. He first was treating me for asthma and then just this past month did an asthma test which was negative. He says I have PAH and I said shouldn't I be seeing a specialist???? He said my PAH was very mild and was not necessary. In the meantime the EP did my ablation in February and has now basically discharged me with "come back in a year." I said what about this PAH issue? and so he referred me to a regular cardiologist in the practice that "is the only one there that sees patients with PH. In the meantime I requested a second echo which was done in mid-April which showed lower pressure (41). He says I "don't need a right heart Cath" and my pressure is "very mild" , come back in 6 months
but he "doesn't know what is causing my shortness of breath". He did agree to prescribe the sildenafil however basically at my request and let me see if it does anything. After only 3 days on it, I think I can say yes it is making my breathing easier.

My difficulty is how to take it - prescription only says "3 times a day" - how many hours do I space it out? Reading about it on line says it only lasts about 4 hours, peaking in 60 minutes, but I wonder if that is if you are taking the higher dose for erectile dysfunction. Does it stay in my system for PAH longer than that? Does it matter if I take the last dose before bed or not? Do I need it overnight? So many questions......guess I need to go have a chat with the pharmacist.