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Pulmonary Arterial Hypertension now a possibility

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#1 mando621


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Posted 09 January 2014 - 10:48 AM


It has been a long time since I've been on the forum site. I have been dealing with things quite well, at least I thought I was.


I have a diagnosis of Undifferentiated Connective Tissue Disease with possible Sjogrens and Scleroderma. I was recently at my rheumatologist appointment when I mentioned that I had had a very irregular heart beat during my colonoscopy. My primary care doctor had ordered an echocardiogram which he then said came back fine and not to worry about things. I have been having slight breathlessness as I hike around campus to class and recently had to tell my daughter to walk slower when we were out together. Usually they were telling me to slow down.


The rheumatologist looked at the echo report, and said that it indicates that there is a possibility of pulmonary hypertension, pulmonary arterial hypertension. He has ordered another echo and PFT tests. My last PFT was 2 years ago since last year the rheumatologist didn't think I needed one. Also may end up with heart cath if needed. There is one pulmonologist at the hospital I go to that specializes in PH/PAH so if I need a heart cath I'm going to see if I can get a referral to him.  My rheumy said that my primary care doctor wouldn't have known what to look for.


Anyway, this is really getting to me. I have come to terms with most of the issues I have and I really felt that I was managing well. I have very little as far as skin changes, just some tight facial skin and smooth fingers and palms. I don't want to jump to conclusions just yet, but this is quite a blow.  While I have escaped many issues that a patient with scleroderma must deal with, I may have an issue that changes the whole picture.


So, thanks for a little venting. Also, make sure that you see your rheumatologist regularly and that they know connective tissue disease issues or this kind of thing could go unnoticed.



#2 Joelf


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Posted 09 January 2014 - 01:35 PM

Hi Mando,


I'm so sorry to read your post and I do hope that your diagnosis of Pulmonary Arterial Hypertension will not be as bad as you fear. Unfortunately Scleroderma does have a habit of dealing you an unpleasant blow, when you least expect it!


Thankfully, I've not experienced PAH, so can't advise you further myself, but please do update us with the results of your Pulmonary Function Tests (PFT's),ECHO and also the heart catherisation.


Kind regards,

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#3 miocean


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Posted 09 January 2014 - 02:42 PM

Hi Mando,

First of all, your doctors are doing the right thing by testing you for pulmonary arterial hypertension. The latest research shows that any scleroderma patient who presents with shortness of breath should be screened for PAH. The gold standard is a right heart catherization to diagnose so you will more than likely have that procedure. During the right heart catherization they will test the pressure between your heart and your lungs. It if is >25 it is diagnosed as PAH. 


It was suspected I had PAH four years ago based on my Echo and medication was suggested to treat it. While I was waiting for approval for the drug I received a kidney transplant and my nephrologist didn't want to add any more medications to the anti-rejection ones so I did not take anything. My next Echo did not show high pressures. The Echo a year later did so I had a right heart catherization in the spring and my pressure was 43. I was put on medication and had a left and a right heart catherization in September and my pressure was down to 23 (Yea!). I have a pulmonary hypertension specialist now beacuse, like scleroderma, it is a rare and misunderstood disease. The drugs to treat it are very expensive so good insurance is a must. Interestingly, Viagra and Cialis have been found to help lower the pressures as they are vasodilators. More serious cases are treated intravenously with pumps.


Try not to worry (says the Queen Of Worriers) and think positively. You are doing all the right things, looking into the diagnostic tests, finding a doctor that is familiar with PAH, and coming here for support and advice. If you are having lung issues you may want to look into a pulmonary rehabilitation program. It was extremely helpful for me. Please keep us updated on what is going on.


And yes, scleroderma does have a way of rearing its ugly head...



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#4 mando621


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Posted 09 January 2014 - 06:58 PM

Thanks Miocean and Joelf, it is reassuring to know that there are people who understand. I really can't talk to anyone around here without it becoming an issue. My husband brushes off most things, and when I finally shared my worries with him, he understandably said don't worry until we know for sure. So, next Friday are the echo and PFT. We will see if I get a referral after that for further testing. Looking at my most recent echo in September had a few warning signs if you look at it with connective tissue disease in mind. Bowing of the septum, pericardial effusion, right ventricle pressure 42. I have a small hole between atrial chambers. Slight regurgitation in mitral valve. The cardiologist that read the echo didn't think it was significant, but my rheumy said that this is missed if you aren't familiar with CTD.

I met with my knitting group tonight. The big topic of discussion was my daughter, 23 yr old, that recently went through surgery to remove a cancerous colon polyp. If stress makes this stuff worse, I've had a lot lately.


#5 Amanda Thorpe

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Posted 13 January 2014 - 01:22 AM

Hello Mando


I am sorry you have this hanging over your head! As it happens an ECHO can only suggest the presence of PH/PAH but a right heart catheter test is required to give an accurate diagnosis as the catheterisation measures the pressures in the heart. A member of these forum was told they had PH on ECHO, when they had the catheter test it showed that they did indeed have PH but it was much less severe than suggested by the ECHO.


Take care.

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