CREST systemic sclerosis

[Joan59]

spent the day at the Sheffield hospital yesterday having a heart catheterisation and when all results were in the consultant said I have developed pulmonary hypertension on top of my Scleroderma. Don’t know how I feel about this yet.

[amc282]

Joan, take one day at a time. x

[OldTed60]

I’m so sorry you’ve developed PH on top of limited systemic sclerosis. It will probably take a while to process but hopefully targeted treatments will help keep it under good control.

Hope you don’t mind me asking if you have had symptoms prompting the guided catheter? I’m not near a specialist scleroderma centre such as Sheffield but told I’m high risk too - but can’t even get an echocardiogram done annually here - only had one 2 and half years ago and it seemed basic - no catheter or anything. But my annual PFTs are always fine so far and I don’t have much breathlessness or lung issues so far. So my rheumatologist says she will chase up annual echo but to just let her know if I become symptomatic and she will make sure I get one urgently. Afterwards I wondered what symptoms I should look out for other than increased breathlessness?

[Bkart in reply to OldTed60]

Hi OldTed, I think PH can sometimes be asymptomatic, my results are borderline. Every echo I had always showed PH so I had a right heart catheterisation. My symptoms are breathlessness on exercise and particularly when bending unfortunately when I had the RH cath., I had to stay in bed for five hours before the procedure so it was unclear. My cardiologist suggested I needed a specialist exercise induced RH catheterisation but have never been given one. The breathlessness has got worse and just daily routine activities around the home can now set it off. Just waiting the results of annual LFtests, this time I had to do a six minute walk test which at my hospital is now done on a treadmill. Not sure what it will reveal as I was so breathless they stopped the test before completion. I think you should not only get an echo but also your Rheumatologist should request a six minute walk test for a thorough evaluation.

[OldTed60 in reply to Bkart]

I didn’t want to like your reply Bkart because it sounds horrible but thanks so much for letting me know what to look out for. My rheumatologist isn’t a scleroderma specialist - although I know that Sjögren’s too can cause PH so double likelihood for me I guess.

But annual LF/ PFTs tests have so far been in normal range so I guess that this and normal echo 2 and half years ago are why the annual echocardiograms haven’t materialised yet. Prof Denton did say in July that both tests are important to have annually and my rheumy agreed at my review on Tuesday and will tell cardiology to pull up their socks and test annually from now on.

I’m not too worried from what you say though as I’m not that breathless most of the time - just a bit out of breath and very weary and slow walking uphill - but assume this to be just fatigue. Always been fine on treadmills previously when I had testing last year to exclude POTS and PAD - and keep myself quite fit generally walking our dog. The uphill issue isn’t new for me. Down hill is even more scary for me with numb feel and severe disc degeneration - balance lousy! But really nothing lung wise on what you describe xx

[Joan59 in reply to OldTed60]

Thanks for the reply. Good news you don’t have this issue. I have had my condition for over twenty years so it has taken this long to manifest. But I’m a positive thinker and will take whatever comes my way. All the best.

[purpleknitter99]

I have Crest and Sjogrens.. Have mild chronic heart failure in left side and left ventricular tachycardia pacemaker induced . Am 100pc paced so left ventrical getting thicker trying to keep up with the paced right. Recently the cardiologist heard a P2 heart sound which can indicate pulmonary hypertension. If I have that I will eventually get right sided heart failure ! So am on a hiding to nothing !! Fun and games !!