Pulmonary Hypertension

[spire99]

I'm a 29year old female with no prior history of lung or heart disorders. I've never smoked. I've always been extremely athletic. Before April 2010, I would work out 4 hrs a day doing varies training regiments. I would run over 20miles/week plus train muay thai and rock climb. I'm 5'6" and I've always been around 130-135lbs.
In April 2010, I was unable to walk to the kitchen w/SOB, chest pains, dizzy, HR <46 and fatigue. Two weeks prior to this onset I gained 20lbs and I was still working out. I saw a cardiologist, my stress test showed oxygen levels < 88%. I had a PFT test, which showed decreased DLco (77%) and lung volumes (86%). The pulmonary specialist told me that my lung volumes are too low for an athlete. My echo showed increased pulmonary pressures (20-30mm/Hg) in 3 months. I have mild mitral, trucspid, pulmonic value regurgitation. My pressures where 46mm/Hg during exertion ~mild pulmonary hypertension.

Recently, my oxygen saturation has gone down to 81% from 99% from sitting to walking 10ft. My oxygen levels vary and when it drops,feels like my heart pops.
Tests thus far are negative: methacholine challenge, VQ scan, bubble test, Chest X-ray, CT scans, no sleep apena, connective tissue disorders. A year prior, I had muscle tissue death. My recent pulmonary function test showed no improvement in my diffusion capacity or lung volumes and some decrease in spirometry.
My symptoms: dizziness, fatigue, confusion, disorientation, swelling in lower extremities (exercise), chills, chest pain, tingling in left arm up to neck and jaw, severe muscle cramps and spasms, nausea, sometimes vomiting, and extreme SOB. My symptoms used to only last 2hrs after exercise and now have lasting any where from 2hrs to the whole next day.

Should I be worried about the pulmonary hypertension? Why the sudden drop in oxygen,is it related to the PH? Why do I have such severe symptoms that are interrupting my life if the PH is mild? What other tests should they do?

[CCFCardio MD2]

I'm sorry to hear you've been having such difficulty.  Your situation sounds very complex as well as frustrating.  First let me state that it isn't clear one unifying diagnosis can explain all your symptoms.  Given your young age and multitude of symptoms, the first thing that comes to mind is lupus.  I imagine your physicians have tested for this, but all efforts should be made to rule this out.  Make sure your ANA and anti-dsDNA are negative.  Lyme disease can cause some of your symptoms, but it would be uncommon for them all to be attributed to Lyme.  Babesiosis if severe and untreated can certainly cause a multitude of symptoms including many you are having.  However, I doubt babesiosis is the problem.  If you truly have pulmonary hypertension you need a CT scan looking for pulmonary clots, a right heart catheterization to measure your pressures, and possibly a biopsy if something like sarcoidosis is suspected.  Again lupus can explain most of your symptoms so look there first.  Given your weight gain make sure your physician has tested you for things like Cushings disease and PCOS.  

Your situation sounds frustrating.  Try to find a physician you like and trust that you can work closely with.  

Best of luck.

[spire99]

added to last post

3 years ago I had a echo stress test at the New Mexico Heart Institute (same place I just had the test done, so it shouldn't be an uncertainty problem in the equipment.) and it showed no pulmonary hypertension (same altitude).

Could the carnitine deficiency cause the hypoxia I'm having during and after exertion?

Some advice is greatly appreciated! I'm trying to get my PhD in Physics and this health stuff is making it hard to function on a normal basis. Thanks!

[spire99]

My ANA and anti-dsDNA are negative. I've had CT Scans for blood clots and those are negative as well. The cardiologist I'm seeing doesn't want to do a heart catherization because he said at the average Joe would have mild pulmonary hypertension at this altitude. I will discuss this with him at my next apt.

I'm seeing a neurologist as requested by the cardiologist and he found my carnitine levels are low and my ammonia is high. Could this explain the mild pulmonary hypertension which in turns explains the decreased diffusion capacity? From what I read carnitine deficiency can cause heart problems,  rhabdomyolysis (hospitalized in 2009), cognitive problems, and the muscle weakness occurring after working out.
Not sure it can cause pulmonary hypertension though. Maybe, it's causing other heart problems not found by the cardiologist?

[spire99]

Thanks. I'm definitely not satisfied and quite frustrated. I will look into these websites you suggest. I've seen 4 cardiologists at the New Mexico Heart Institute, two pulmonary specialists, 3 endocrinologist, several neurologists, two Lyme specialists, gastroenterologists at the University of New Mexico (medical hospital), and the list goes on and on.
I'm not sure I've found any doctors who are capable of looking into this and I'm not sure who else to go to at this point. I'm exhausted all of my resources. Thanks again for your help and advice.

[yarrow]

If you didn't have pulmonary hypertension three years ago and do now, then you have a developing case of pulmonary hypertension.  Personally, I would not regard this as benign.

I'm not trying to be snotty, but it sounds to me as though your docs are mystified, to say nothing of not coordinated with each other.  Again, it would be good to have somebody really capable looking over the whole situation.

Is there any possibility you could get connected with a medical school or a  major center like the Cleveland Clinic?  There are ways to contact specialists directly rather than working through this bulletin board, for example on this page for the Cleveland Clinic's department dealing with pulmonary hypertension:

http://my.clevelandclinic.org/pulmonary/pulmonary_hypertension/physicians.aspx

You'll note that you can submit a question online there.  Same for the Tomsich Family Department of Cardiovascular Medicine:

http://my.clevelandclinic.org/heart/about/specialties/cardiovascular_medicine.aspx

Please think about pursuing evaluation and treatment outside the resources you have been using.  I would not be any more satisfied with the help and information you have received than you are!

[spire99]

Yeah, I wish someone would get back to me soon.
I forgot three years ago (April 2007), I had severe chest pains and SOB while working out that got worse and worse over time but only during exercise. The nurse gave me inhaler and told me I had sports induced asthma (never did any tests). The inhaler never worked and the symptoms of chest pains got worse. I was finally sent to the ER where they found the mild mitral valve regurgitation and was referred to a cardiologist. Several tests were done including the echo stress test (tilt table, bubble test, stress). All of it was negative three years ago. The cardiologist I'm seeing now told me on Tuesday that the average Joe at this altitude (4500ft) would have mild pulmonary hypertension. If this correct, then why three years ago I didn't have it?

[yarrow]

It's too bad that a physician in this forum hasn't posted a response to your question.  This appears to be a complicated medical situation in great need of intelligent, coordinated management.

[spire99]

Hello yarrow,

thank you for posting!
Yes, the Lyme specialist has given me quite an extensive explanation on all of these co-infections. Yes, I was treated in 2008 for Babesia with antimalarials (Mepron) for 2 weeks but I couldn't continue the treatment because I had an allergic reaction  to the medication. I was also treated for Bartonelli with antibiotics for 2 years. In January of 2010, the lyme specialist finally started treating me for Lyme. I started having digestive problems in the mid to end of March and  I stopped all treatment because I was vomiting every time I ate. After stopping the treatment, I started gaining weight and then 2 weeks later I started having the symptoms I discussed above. My Lyme specialist is the one who told me to see a cardiologist to get the okay on my heart before going back on the Lyme treatment. Well 8 months later I'm still being tested. It is a high probability that the Lyme or co-infections are causing these problems but to get the cardiologist or pulmonary specialist to even consider these infections as the root of the problem is a problem.

I saw the cardiologist yesterday and he doesn't think my problems are related to the mild pulmonary hypertension and doesn't want me to worry about it. He also doesn't think it's a heart function issue. He has me on an event monitor for 4 weeks and hopefully that will give him some information.

He wants me to see a neurologist and reumatologist because he thinks it's a muscular disorder which is affecting my heart. I'm not sure whats going on and I'm getting quite frustrated with all of these specialists.

[yarrow]

Neither can I diagnose, but if you have been tested and found to have Babesiosis, you probably already know that although about 25% of those with this infection have no symptoms and recover nicely, there are a few people who become quite ill--and those who have both Babesia and Lyme Disease are sicker yet.  In these folks, pulmonary edema is possible, along with hypoxia, and several cardiac and kidney problems.  These things are more likely in those who have *had their spleens removed* or are immunocompromised in other ways.

You say you were given antibiotics, but were you also given antimalarials?  And for how long?

Here is more information:

http://emedicine.medscape.com/article/780914-treatment

Bartonella also can induce serious cardiac problems:

http://www.thedoctorsdoctor.com/diseases/bartonella.htm

In addition to seeing a cardiologist, you might want to see an infectious disease specialist right soon.  It might be possible that you have a chronic or relapsing disease which happens to be affecting your heart and other organs.

[spire99]

Thanks for posting! I see the pulmonary hypertension cardiologist today and will ask him about my heart function. However, he told me everything looked normal but all he looked at was the stress echo test. I've been tested for pulmonary emboli and it's been negative. My pulmonary specialist said that it's possible it's a precursor to a lung disease that hasn't shown up yet on the CT scan. She just doesn't know which disease it is yet.
My ejection fraction is 60-65% as of August 2010. I think in April 2010 it was around 85%. Not sure if that matters because the doctors tell me it depends on the equipment and the uncertainty, so as long as it's normal that's all that matters to them.
My symptoms are the worst during any physical activity and the weirdest part is sometimes I have good days where I feel some what normal, well until I exercise.
I've also been diagnosed with chronic Lyme Disease, Bartonelli, and Babesia in 2008 and was on antibiotic treatment. Not sure if it's related. Also, seeing a gastroenterologist because I've been having blood clots as well. Also, had a benign tumor removed off the side of my face. All of this all started at the same time. Not sure what's going on.  

[is_something_wrong]

Agree with yarrow, this post is almost screaming some circulation problems. I don't know when the doctor will answer you (it depends) but I would recommend that you see another cardiologist as soon as you can.

I can't give you any suggestions for a diagnosis here (I'm not qualified to do that) but the doctor may look at things like pulmonary embolism (most likely ruled out with CT scan) or heart muscle infection (is your ejection fraction normal?)  Not sure, but you should see a cardiologist or an internist for a second opinion. This is not normal.

[yarrow]

If your symptoms and test results are as you have reported them, I think you need to see another cardiologist--or two.   The pH is not the issue here.  Heart function is.