exercise induced idiopathic pulmonary artery hypertension

[Bluetiger]

I live in Australia,I am 39 years old. I was diagnosed five years ago with complete heart block and received a dual pacemaker. Around six months ago I began suffering terrible fatigue, breathlessness, and pounding heart with little to no exersion. I had a stress test done which uncovered post exercise pulmonary artery hypertension. 22-37 mmhg at rest and elevated to 58>mmhg post exercise,the conclusion, significant post exercise pulmonary artery hypertension.A lung function test showed my DLCO ml/min/mmhg at 16.7, significantly impaired. I have quite obvious cynosis and coldness of my hands, sometimes my feet and face. They fiddled with my pacemaker and concluded after a further stress test (without stress echo), that it was not IPAH?? even though post exercise IPAH was apparent. This was four months ago, and I am still suffering from bouts of breathlessness everyday, sometimes without exersion, especially in the mornings. The symptoms have not altered since the pacemaker readjustment, and I am currently not receiving any medical treatment or tests for any further investigation for IPAH, and I am worried that this disease may be progressing without anyone knowing, or seeming to care.I have been taking 100 mg of metoprolol twice daily for about three years now, for an underlying atrial arrhythmia.Is my concern warranted?? Should I be pushing for further tests?? I hope you can assist me with some advice.
Kind Regards, Bluetiger  

[Forum-M.D.-ppb]

Absolutely.  There are enough concerning signs from your description that your condition must be figured out.  I, as well, am concerned that it may be progressive in nature.  You should definitely see an experienced cardiologist who can help you figure things out.  The cyanosis is quite concerning and if true typically points to a shunt in the heart or the lungs.  The low DLCO points to the lung as a possible cause.  Someone experienced should review your echocardiogram to look for evidence of enlarged heart chambers, infiltrated heart muscle or valvular disease.  Those must be ruled out.  It is also concerning to me that you  have had a pacemaker since the age of 34.  What was the explanation given to you as to why you had complete heart block.  Did they do a thorough workup looking for  conditions such as sarcoidosis, scleroderma, dermatomyositis, lupus or rheumatoid arthritis.  These conditions affect the lung and the heart.  Also, atrial arrhythmias can cause transient sinus node arrest which might look like complete heart block and lead to pacemaker placement.  I am not sure that that's what happened to you.  The fact that you are having arrhythmias lets me know that this condition has been going on for quite some time.  A CT scan of the lung would be helpful as well.  Again, I encourage you heavily to seek  a second opinion and/or further tests to figure out what is going on.

[Bluetiger]

From Bluetiger.

I forgot to say in my post that I am female. (sorry) Thank you for replying so quickly. In answer to your questions, no there was no 'reasons' that they could tell me that I had complete heartblock. And there was no further tests done reguarding scleroderma arthritis etc. The only other issues I have had is a loose dianosis of fibromyalgia, with severe carpel tunnel which came on very rapidly, and was relieved with surgery to both wrists.if this is a progressive IPAH, would the calcium blockers I am taking be helping with the severity??

[Cleveland Clinic]

There are only a few conditions that cause bilateral carpal tunnel syndrome.  Of course the carpal tunnel itself could be affected in both risk, but more importanly one should definitely rule out an infiltrative disease called amyloidosis.  The other condition that could cause this is hyperparathyroidism.  I don't think the latter one is worth pursuing at the point, however, the amyloidosis can be associated with other connective tissue disorders which I described to you before, again, making me think that all that is happening to you is part of a syndrome.  The conduction abnormalities in your heart could also be related to that as well.  Make sure you get a thorough evalution by your physician.  Don't leave any stone unturned.  The calcium channel blockers can sometimes help.  There are two ways to find out.  One is with another catheterization and a direct challenge with that drug to prove responsiveness of the pulmonary vascular tree.  The other, is to just start taking it and see if there is any symptomatic improvement.