Hughes Syndrome and Pulmonary Hypertension with Tricuspidalisinsufficiensy

Someone else with this issues?

Kerstin in Stockholm

20 Replies

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  • Pulmonary Hypertension awaiting further tests to see if I need to go to a specialist hospital. Do not have much of problem once I am rested but doing the simplist things can make me gasp for air

  • Thank you for your answer!

    What number is your pulmonary hypertension? Mine is only 45-50. Have they done an Ecogardiography on your heart/lungs? Did you also have any valve-problems?

    Kerstin

  • My first one in 2012 was a little lower than that but I have other heart and lung failure to make it a touch more complicated to deal with. I have had tests were they say there is a valve issue then another saying there may not be. To date I have had 8 cardio versions and 3 ablations to try and sort the heart out but the Ablations have now scared the chamber of the heart I had 38 burns over7.5 hours before they decided it was too much and I was still sparking in the wrong places? I only have 54% lung function upright and lose a further 12% when flat! I am losing approx 3 % lung function a year. The problem in my area is we do not have a PH specialist. Even my consultant admitted he knows very little about PH and I am having some more tests next week to see if I can get an appointment at a PH centre which is 70 miles away from my home. All he will say is I am ticking all the boxes? I have been deemed exercise intolorant.

    Be Well

  • Thanks! Probably we have different problems. I understand very little about it and I am not sure if I want to know too much either. I am happy because I can exercise and I have not been breathless yet. I have not done a right heart catheterization as they do not want to go inside my body if not necessary as to my 3 antibodies in high titres. I have done 7 Ecocardiographies with doppler.

    I am lucky as I am probably at the best hospital in Sweden and have the best Doctors and I selftest with a high INR which I have understood is very important. They also understand what APS is. Does your Doctors understand that we have too thick blood?

    When were you diagnosed with Hughes Syndrome and can you stay at a steady and high INR today?

    Be well!

  • Hope you are ok? Let me know if you want me to find any papers or anything, I know you keep yourself as fit as possible. MaryF

  • You are so kind Mary! Thank you, but i just wanted to know if I was quite alone with this "fenomen". I feel fine as usual.

    I do not want to know too much too early as it is diffucult to jugde from the outside and it could even frighten me. I just continue to keep my INR in range if possible. That is all I can do.

    Kerstin

  • xxx MaryF

  • Hope your okay💕Thinking of you

  • Hi Cindy;

    Hope you feel ok with your new anticoagulation!

    I am quite ok I just wanted to know if there was someone else out there with this uncommon symptom of Hughes Syndrome.

    Thanks for your hearts! I wonder if I also have those figures on my computer!

    Kerstin

  • They are in the smiley face at the bottom of my screen. I tap on the smilee face and it gives me options. Very glad you are well. Love love love the enoxaparin! Stay well

  • I hope things go well and i am also glad you can still exercise!

    Gina

  • Thank you! We have a ev terrorattack here in Stockholm just now so people are trying to get home.

    Love from Kerstin

  • Hope all remain safe, this world is so scary sometimes

  • Aloha from Hawaii. ..

    I don't have pulmonary hypertension, but do have regular hypertension and have had aortic valve replacement and of course APS/lupus.

    Occasionally I get a flare which causes the pleural sac around lungs to be painful....

    I am managing everything pretty well.

    Hope you are ok!

    Kerstin from Hawaii

  • Thank you Kerstin!

    I am fine but please try to keep your bloodpressure down.!

    Kerstin

  • Hi Kerstin,

    I have 3 antibodies as well and was diagnosed with Pulmonary Hypertension with a definitive right heart catheterization 1 1/2 years ago. I have also had 2 VQ scans to verify micro clots in my lungs. I have also had 3 cardiac ECHOs done every 6 mos over the past year. I also had a nasty clot that looked like algae in my Trans Esophageal Echocardiogram hanging off of my aortic valve. This all occurred about a year ago. I also was unable to walk up the stairs or down the street without resting.

    Today, luckily, I am feeling much better. I am trying to maintain my INR at 3.5 or higher (the doctors here in Chicago are still skittish about going higher, but I have a Coagucheck so I keep myself on the high side towards 3.8 to 4.0) with 7.5 mg to 10 mg of Coumadin daily. And if I dip below 3 I give myself a shot of Lovenox. I also am taking a new drug (2013) that has been helpful opening up my vessels blocked by micro clots in my lungs it is called Adempas and it is made by Bayer.

    From their website:

    Adempas (riociguat) tablets is indicated for the treatment of adults with persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH), (WHO Group 4) after surgical treatment, or inoperable CTEPH, to improve exercise capacity and WHO functional class.

    Adempas is indicated for the treatment of adults with pulmonary arterial hypertension (PAH), (WHO Group 1), to improve exercise capacity, WHO functional class and to delay clinical worsening.*

    Efficacy was shown in patients on Adempas monotherapy or in combination with endothelin receptor antagonists or prostanoids. Studies establishing effectiveness included predominantly patients with WHO functional class II–III and etiologies of idiopathic or heritable PAH (61%) or PAH associated with connective tissue diseases (25%).

    *Time to clinical worsening was a combined endpoint defined as death (all-cause mortality), heart/lung transplantation, atrial septostomy, hospitalization due to persistent worsening of pulmonary hypertension, start of new PAH-specific treatment, persistent decrease in 6MWD and persistent worsening of WHO functional class.

    I am able to exercise now: ride a stationary bike for 45 minutes and walk 3 miles.

    I also take an aspirin daily , and Diltiazem 120 mg daily, I also use oxygen for at least an hour before I go to sleep at night and sleep at Zero Gravity (raised head and raised feet) which has helped my peripheral circulation. I had an ECHO done of my heart yesterday and my Aortic Valve clot is gone and my partial pressures in my heart are normal. So the therapy is working. I just took a trip to Taos, New Mexico at elevation of 6,700 feet (over a mile up) last week and had some difficulty breathing but used a mobile oxygen condenser, that I take with me on trips, at night to sleep. My pulmonologist here in Chicago at Northwestern University Hospital is Dr. Michael Cuttica phone number (312 695-4965)

  • Hi and thank you very much for your information!

    I am glad that it works so well for you now and that you must have great Doctors who is treating you.

    I wonder if you have had very high bloodpressure earlier and have they mentioned your Tricuspidalisvalve ever? The problem is my right heart chamber. If I get worse and have difficulties to breath they must do a right heart catheterization. As it is now they are not quite sure of my status I think.

    Keep well and thanks again for your good answer!

    Kerstin

  • K,

    My blood pressure did go up before I was treated with Adempas. Today it is back down to 113/54. My tricuspid valve is Ok some minor regurgitation. The big issue for me was my aortic valve bc a clot was hanging off of the valve. Luckily it has been resolved. The Adempas has really helped my Pulmonary Hypertension. Don't know if the micro clots in your lungs ever really go away but Adempas helps the vessels to open up and allow blood through. Pulmonary Hypertension is bad for the heart. The heart will enlarge because it needs to work harder to push blood through the lungs when there are microclots occluding the flow. The heart catherterization is the definitive test to measure right ventricular partial pressures and determine whether Pulmonary Hypertension is present. They can estimate right ventricular systolic pressures from an ECHO 2D M-Mode C/Full Doppler.

    I monitor myself with a pulse ox that I purchased on Amazon. It lets me know my oxygen percentage in my blood and my pulse. Access to oxygen is also nice. Keeps the headaches and muscle spasms from occurring.

    hope this helps,

    A

  • Thanks again!

    I know about the right heart catetherization and I believe they will do it if I get worse and they have told me to at once report if I feel breathless.

    I have good Specialist-Doctors at my hospital but the Tricuspidalis-valve is an unusual valve to have complications with our illness and I am not young anymore (73 in June), triple-positive and persistant high titres of the 3 antibodies.

    I believe it is the high and stable INR that keeps me going just fine so I will stick to that regime. I did an Echo the other day and my Rheumatologist is very interested in heart-issues together with primary APS (me) so I will discuss what you have told me also very soon!

    Thank you again for your very good info and I wish you a Happy Easter without too much cholesterol perhaps (we eat a lot of eggs for Easter in Sweden).

    Kerstin

  • K, Happy Easter, 🐣 at 54, I hope to make it to my 70's! You are an inspiration. Thank you for all that you do!

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