Pulmonary Hypertension

[erikainorlando]

I just received an email from a woman I have been correspondeing with who I have always thought sounds like she has symptoms of POTS....but she is saying she thinks she may have pulmonary hypertension....has anyone heard of this?

Is this similair...just checking...I know you all aren't docs but you sure have a lot of info!!

Thanks,

Erika

[firewatcher]

A la Wikipedia:

In medicine, pulmonary hypertension (PH) is an increase in blood pressure in the pulmonary artery, pulmonary vein, or pulmonary capillaries, together known as the lung vasculature, leading to shortness of breath, dizziness, fainting, and other symptoms, all of which are exacerbated by exertion. Pulmonary hypertension can be a severe disease with a markedly decreased exercise tolerance and heart failure. It was first identified by Dr. Ernst von Romberg in 1891. According to the most recent classification, it can be one of five different types: arterial, venous, hypoxic, thromboembolic or miscellaneous.

Because symptoms may develop very gradually, patients may delay seeing a physician for years. Common symptoms are shortness of breath, fatigue, non-productive cough, angina pectoris, fainting or syncope, peripheral edema (swelling of the limbs which commonly manifests around the ankles and feet), and rarely hemoptysis (coughing up blood). Pulmonary arterial hypertension (PAH) typically does not present with orthopnea or paroxysmal nocturnal dyspnea, while pulmonary venous hypertension typically does.

In order to establish the cause, the physician will conduct a thorough medical history followed by a physical examination. A detailed family history is established to determine whether the disease might be familial. A history of exposure to drugs such as cocaine, methamphetamine, alcohol leading to cirrhosis, and smoking leading to emphysema are considered significant. A physical examination is performed to look for typical signs of pulmonary hypertension, including a loud P2 (pulmonic valve closure sound), (para)sternal heave, jugular venous distension, pedal edema, ascites, hepatojugular reflux, clubbing etc. Evidence of tricuspid insufficiency is also sought and, if present, is consistent with the presence of pulmonary hypertension.

Looks like a very specific condition that is readily provable if given the right tests.

[all4family]

Hi Erika,

I have heard of it. It is fatal, so if she really thinks she has it, she should get it checked. I thought I did for a while, and people with Pulmonary hypertension get Orthostatic Intolerance secondary to the pulmonary hypertension. I have heard of one woman who had a surgery, that improved her status with it, but the surgery only works a small percent of the time. This is what I have learned about this myself, and of course I am no doctor. I hope your friend gets this checked out.

Suzy

[erikainorlando]

Thanks guys! It sounds very serious. Symptoms do sound overlappnig somewhat but doesn't soundike the hr increase on standing.... ??

[all4family]

My understanding is yes it does. I knew someone (online) that was diagnosed with first orthostatic intolerance, then later she found out she had pulmonary hypertension. I don't want to worry anyone on here though. We are some of the most well medically tested people there are. What gave me reassurance I didn't have it (because I was really worried) was that I had had a Echo, and an echo stress test. My ejection fraction was ok, although 3 of my heart valves have small leaks. This person had a predisposed factor though, because she had taken the phen phen pills, and that increases your risk. But if your friend thinks she has it, I hope you will encourage her to get it checked.

Suzy

[Kathy_Ireland]

Do encourage your friend to get this checked if she thinks she may have it. It is a very serious condition but some strides have been made in it's treatment.I recently saw a TV program about a woman who had this illness and was confined to bed. She was treated with Viagra,which is now a common treatment for Pulmonary Hypertension, and it made a very big difference to her quality of life. She was able to get out of bed and live a normal enough family life and go out thanks to it. I'm not sure if viagra or any treatment can impact the over all prognosis but maybe it might help your friend to hear something a little bit encouraging before she embarks on having it investigated. Good luck to her.

[flop]

If you have a detailed echocardiogram done they often include an estimated pulmonary artery pressure (PAP) - in people with pulmonary hypertension the PAP is very elevated. I think that the right ventricle would also show abnormalities and be larger than normal.

Luckily most of us will have had echos as part of our POTS work-up so don't need to worry.

Flop

[jump]

OK, I read up on PPH and now I'm really worried, but I think I might be being a hypochondriac for real this time, so I could use all your input....

I had an echo done in 2005, but wasn't diagnosed with POTS until 2008. My symptoms had been getting worse during that time. When I was dx, I had a 24-hour holter monitor test and a pulmonary function test, but no other heart tests - no stress test and no second echo.

I'm worried I might actually have Primary Pulmonary Hypertension because I took ephedra (type of diet drug) for several years (probably 1999-2003), which is now banned because it can cause "heart problems." I never bothered to look up what these possible heart problems could be, but when i recently researched it it turns out the "heart problem" this drug can cause is PPH.

Then the other reason that I'm worried is that I have this constant chest-pain that is still unexplained, and I get high blood pressure and breathlessness when I exercise, all of which has been getting progressively worse even though my tachycardia has gotten better. My doc has always attributed this to the POTS.

Part of me tells myself not to worry because if I had PPH it would have shown up in my first echo, right? Even if it was milder then? I'm not entirely sure my doctor knows I have a history of taking diet pills, because she became my doctor after I had already stopped for a few years... I like to think I remembered to tell her, but I'm really not sure.

Ugh. I know I should probably just ask my doctor all this but I'm already trying to convince her to test my adrenal function which she doesn't think I need because my potassium is normal, and she refused to order an echo when I was diagnosed with POTS because I had had one three years previously that was fine. Sometimes I feel like I have to pick my battles with her when it comes to ordering tests, and I don't want to ask for another echo if I'm just worrying for nothing.....

I suppose if I had PPH and not POTS, my hr wouldn't go up when I stand, right?

[erikainorlando]

Jump -

I always enjoy when you post....I have no real understanding of the difference. BUT I wanted to say that please have your PCP test you for whatever you think you need. The way I see it...we are customers...I mean we don't have to be difficult customers but really..most all my doctors will order anything I think I need....I think we deserve that given that we are so disabled so much of the time....I mean it is not like we are suffering from a 3 day flu.

Just my 2 cents

Erika

[flop]

Hi,

I've had a bit of a google for Ephedra - it seems to be a herbal medicine similar to ephedrine so it acts as a stimulant much like amphetamines and ecstasy typically causing increased heart rate, increased blood pressure, increased body temperature and increased sweating. All of those effects increase the amount of calories that the body is using up so it was an "obvious" candidate for becoming a diet-pill.

I've not found any evidence specifically suggesting that pulmonary hypertension is a side effect (as opposed to systemic hypertension which is a side-effect).

It would seem to me that the drug is fairly short-lasting and that the side effects would wear off quite quickly when you stopped taking it. If you stopped taking the Ephedra in 2003 and had your echo in 2005 then I doubt that any effect from the ephedra would have waited more than 2 years before showing up.

The bottom line is that your echo will probably be normal again if repeated now, but you are worried and a repeat echo would reassure you. I don't think that it is unreasonable for you to ask for a repeat echo now (4 years after your first echo). Do you see a cardiologist (many of us with POTS see cardios)? Perhaps you could ask for a cardio referral and then get the cardio to order an echo?

Try not to worry too much, pulmonary hypertension is very rare and the symptoms you describe are very common for people with POTS so most likely are due to POTS.

Flop

[all4family]

Hi Jump,

I just wanted to tell you that I had the hypochondria about this one also. I think one of the tough things about this disease is that it mimics so many others that are very serious. I have had the constant chest pain, and shortness of breath also. (I've decided it was a pulmonary embolism this time! ) I think what flop says is very true if you took the diet drug, then had the echo 2 years later that would give me a lot of reasurance. If it is making you worry though I think I would definatly try and get another echo done. Let them know you took the diet pills, and are concerned due to your symptoms. Don't make yourself sick over this. Just find out. I made mysef crazy worrying about it. POTS really gives us a lot of symptoms, and if your worried it is something else rule it out, so you don't have to worry. Which is worse asking for a test that comes out negative, or worrying about something and causing yourself unneeded stress. Take care, and try not to worry.

Suzy

[Tammy]

I can say that this runs in my mom's side of the family and it's never been something they can't pin point. It gets diagnosed and patients are put on oxygen and meds. The symptoms are a bit different than POTS. I can relate to their weakness and a few of the symptoms but it is different than what POTS patients present with. Hope this helps relieve some worries on Pulmonary Hypertention.

[jump]

thanks guys! I feel better about this now. I'll bring it up with my doc next time I see her just to be sure, but I think probably she'll tell me not to worry about it, and now I won't.