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  • Beverly
    Participant
    Post count: 5

    I AM REPOSTING. I FAILED TO PUT A SUBJECT.

    I saw a supposed PH specialist and was dismissed as having PH. I think my sister, mother, and I all may have been misdiagnosed/undiagnosed and possibly have PH. We all 3 have heart disease and multiple conditions which can be related to PH. Me: lung scarring, previous lung nodules, heart disease, diastolic dysfunction, ejection fraction 50%, thyroid condition, suspected autoimmune connective tissue disorder. Sister: heart disease, diastolic heart failure, sleep apnea, thyroid goiter, asthma. Mother: heart disease, diastolic dysfunction, enlarged heart, right bundle branch block (EKG), right/left ventricle enlargement on echo, asthma.

    I believe I read that it is possible to have PH even if the echo pressure is normal. Is this correct?

    I believe I have read that you can have a normal pulmonary function test if you have mild PH. Is this correct? (I had a recent PFT that was normal – other than flattening of flow volume loops – but I’ve had previous ones with abnormalities. I’ve had one with restrictive defect and one with reduced diffusing capacity in the past.)

    ALSO…

    CPET: A cardiopulmonary exercise test I had a few years ago that showed ventilation perfusion mismatch. One of the things mentioned as a cause of my particular CPET findings (V/Q mismatch) is Pulmonary Arterial Hypertension. I did not learn about this part until several months back. This test was ignored by the doctor.

  • Ava
    Participant
    Post count: 34

    Hi Beverley,
    The only way PH can be diagnosed (or definitely excluded) is via a Right Heart Catheterisation (angiogram). Left heart disease is one of the commonest causes of PH. Serious/long term lung problems are also a regular cause of PH. Ditto auto-immune disease.
    It is very possible (even likely) to have normal lung function tests or echo-cardiograms or ECGs with PH.
    Once PH has been diagnosed, it is normal to undertake multiple further tests (such as VQ scan etc) to determine the cause of the PH – the cause of your PH will determine the treatment.
    I had severe PH when I presented to my local ED, to the point my ECG was showing the arrhythmia I was suffering from severe right heart failure. The doctor told me a lot of people had arrhythmia due to electrical problems in the heart and that I should go home and make a routine appointment with a cardiologist (I was 43 and had no previous health problems). I had to return to the hospital the next day to pick up some paperwork, and some nurses saw me trying to walk (my PH was so advanced I had severe SOB/light-headedness) and practically dragged me back to the ED and insisted a doctor look at me. Luckily, a cardiologist was visiting my rural area that day and he performed an echocardiogram. My right heart failure was so severe it was immediately obvious I had PH – I was admitted to hospital and not allowed out of bed for 3 days until I could be transferred to a major hospital 3 hours away. There I saw a PH Specialist and had a Right Heart Cath to confirm the diagnosis (after lots of tests to rule out other causes, I was diagnosed with Idiopathic PAH). I also found out my liver was failing. I found out later that I was within probably a week or two of dying, my pressures were so high and my right heart failure was so severe. I mention all this to show how hard PH can be to diagnose – I was seriously, seriously ill, and yet the first doctor I saw had suggested it was just a minor problem and I should just go home and wait a month or two to see a cardiologist (if I had, I’d be dead).
    My PH has now stabilised and it would be extremely difficult for a regular doctor to tell that I have PH – even though my pulmonary artery pressures remain in the moderate range. I’ve also learnt to avoid any doctor (even cardiologists) who are not PH Specialists – in the 4 years since my diagnosis I have (by chance) seen different doctors (mainly because they’re interested in examining me and learning about PH). Inevitably they’ve all tried to change my meds or change something that has made my condition worse, not better. I now refuse any medical treatment that hasn’t been cleared by my PH Specialist (luckily he is very accessible via phone or email, even though his practice is in a major centre 3 hours from where I live). The right doctor can literally save your life.
    If you have concerns about PH, your GP needs to refer you for a Right Heart Cath (or find a PH Specialist who will do so).
    Best of luck and let us know how you go.
    Ava

  • Beverly
    Participant
    Post count: 5

    Hello Ava,

    I truly, truly cannot thank you enough for responding so quickly. I do hope that others respond as well, but even just your reply lets me know that I’m not crazy for being concerned about this possibility – and that I should probably seek a 2nd opinion for myself, as well as try to help get my sister and mother evaluated.

    The main thing that I needed to verify for accuracy was if it’s possible to have PH even though you have a normal ECHO and PFT. And you did confirm that. I had done fairly extensive research before I even called or went to the PH specialist I saw, and I thought that it was I read several places.

    Yes, I do know that right heart cath is the only way definitively diagnose or rule out PH. I WAS NOT OFFERED A HEART CATH. My echo didn’t show elevated pressure; THIS PFT was said to be normal; my 6 min-walk test was severe functional impairment at 39% of predicted (which could potentially be related to my other condition). I was told that it could not be PH if PFT and echo were normal.

    I know myself and my family members each have multiple issues that are common with PH. My sister and I have significant symptoms that seem possible to be PH, yet we don’t seem to show much on the echo. Although, I’m not sure her recent echo even showed the pressure. It was specific to look for heart failure, and I didn’t see anything about RA pressure on it at all. My Mom’s echo has it listed clearly. They even have the same doctor so done at the same place.

    Thank you so much for sharing your story. I’m so sorry for what you have gone through. Thank goodness you finally received appropriate care! I am still so stunned that I had this experience with someone who is a PH specialist. Sometimes I think maybe it just isn’t PH with me – yet his reasons for saying it can’t be just don’t add up.

    Regarding my primary doc, he is so busy with so many patients that he is required to see daily due to contracts with the governing hospital that he doesn’t have time for someone as complicated as me. He will likely assume that this PH specialist that I tried is correct that I can’t have it because THIS PFT was normal and echo didn’t show high pressure. He may be willing to provide a referral for ANOTHER PH SPECIALIST, but that’s as far as he’ll likely go.

    Again, I can’t thank you enough for sharing your situation with me. I’ll take all the information and experiences that I can get. By the way, I’m 48 and my sister is in her 50s.

    Beverly

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