pulmonary hypertension

[Karen]

I was concerned about hijacking Bob's thread -- stenosis pathology without valve damage -- so I'm posting in this new thread a few more questions about pulmonary hypertension.

Nancy said that "on this site there are several with PH." Sometimes I feel a bit like Chuck, expressed in another thread -- I have learned so much ("sucking information/support from others" in this forum). As I am way out in the "waiting room," I hesitate to jump in too much, too often, although I am in awe of the wisdom and caring support given to every person as they face their surgeries/tests/ procedures. So I feel almost apologetic to ask even more questions on this subject. My sense is that I shouldn't worry about the "mild pulmonary hypertension" indicated in my last echo. I can't say that I have obvious symptoms -- although, I still wonder how you distinguish the symptoms of SPH from valve issues and, in my case, from chronic costochondritis (and maybe even anxiety).

For the first time in 1 1/2 years, I had an indepth discussion with my registered nurse daughter about my valve issues etc. After reading the articles about the heritability of BAV, I really wonder about having my adult children screened for BAV. This was what prompted our discussion last week. She was a lot more concerned about the pulmonary hypertension than any of the rest of the stuff with my valve. And with Nancy's comments in the other thread, I still don't know what attention I should bring to this with my cardiologist. I'm sure I'll wait until my next scheduled appointment with him... But I feel like I've learned ALMOST enough from some of these recent posts to ALMOST get a handle on what is prudent...

I'm not even sure what questions to ask. But it seems that if secondary pulmonary hypertension is resolved when the cause is corrected, there is nothing that needs to be done during the waiting for AVR surgery? And yet, does that make sense? Are there any long term effects of this?

I would be very interested in hearing from some of you that have PH -- especially secondary pulmonary hypertension. Maybe YOU can tell ME the questions I want to be asking you, or my cardiologist. I just need some perspective here. And probable reassurance that I need do nothing for mild PH. Or do I? I have been so grateful to get OFF all medications (except for ulcer stuff), that I certainly don't want to volunteer to take something that isn't clearly warranted. But I am haunted by the suggestion that EARLY intervention is important. Even in SECONDARY PH?

Any significance to LEFT Atrial enlargement? Is it just part of the package with the PH? For the most part, I've only worried about ventricular enlargement etc.

Thanks for any light any of you can shed on these questions!

Karen

 

[Nancy]

Karen-

What I have learned from the Pulmonary Hypertension Association website and from Joe's experiences is that ANYONE with a diagnosis of PH needs to be seen by a specialist in the disease. believe me when I say that not every cardiologist or every pulmonologist is a specialist in the disease. It is too rare a disease and most will NEVER in their careers see another patient with the condition. Do not rely on them for the final answers on this problem. They may not be up to speed on what treatments are available or how to proceed.

So an evaluation from a specialist is of utmost importance, especailly in the beginning. Echocardiograms are almost useless in the diagnosis of PH. They can be wrong in either direction. The GOLD STANDARD for testing PH is the right heart cath. which is an invasive procedure. But it is with this test that the pressures can be measured with accuracy and if PH is present and there is a specialist in the room, tests will be done on what works in your own particular situation to relieve the symptoms. It's called a drug challenge.

The evaluation of primary or secondary needs to also be done by a specialist in the condition. They always thought that Joe's was secondary, but it was not. His was primary.

Of course, with secondary, it is very important to find out what has caused the PH in the first place so the cause can be taken care of.

There are not specialists all over. Most people will have to travel to one to get an evaluation.

It may very well turn out in a lot of cases that someone does not have PH, but that is what the specialists do.

If it turns out to be bona fide PH, there are several very good treatments available now. Getting it treated early on makes the most sense.

The waiting time for most specialists is approx. 3-4 months. Most would want to do their own testing, But they will tell you what they need.

I can tell you that Joe's left atrium is enlarged. Two and a half years ago, both the atria were enlarged. His pressures have gone from 75 down to 44 at the highest with Tracleer.

 

[Nancy]

The PH Assoc. website.

http://www.phassociation.org/

 

[Karen]

Nancy, thanks!

Nancy, thanks!

for the quick response... I HAVE checked out the PH website, and there's one PH specialist an hour north of me -- been treating PH for 22 years and works with 5 other PH specialists. Would I ask my cardio for a referral, or look into on my own?

You said, If the condition is Secondary, it's important to find out what caused it in the first place... I have just assumed that the stenotic BAV CAUSED it. Not necessarily so? That's why I am interested in finding out if there aren't a LOT of BAV folks here in the forum who also have "mild PH" just as a matter of course with the BAV. Of course, Betty suggested there was a stronger connection to the mitral valve and PH. I have mitral regurgitation, but nothing indicated about mitral valve abnormalities.

Also, does it matter if you are symptomatic or not? Like I said, I don't THINK I am, but the costochondritis stuff is a little tricky. I suppose the tests done would give me a definitive answer about that, too?

Thanks so much for your post. It's very helpful!
Karen

 

[Lynlw]

Karen, I always, just called the other doctors myself for Justin. I was curious tho if you don't mind, do you see an adult congenital heart cardiologist or a for lack of a better word regular cardiologist? Lyn www.caringbridge.org/nj/justinw

 

[Nancy]

Karen-

Your best bet would be to call the specialist's office and see if someone there even thinks you need to be seen, then let them take it from there telling you what you have to do. I suppose your insurance would dictate how to go about it as well.

I just made Joe's appt. myself, online, since it was an emergency and his former cardiologist didn't have a clue. He was seen within the week and admitted to the hospital for a month. He was very, very ill.

 

[tammy70]

Ph

Ph

I have a BAV and have been diagnose with pulmonary hypertension. I have mitral valve prolapse and insufficiecy too. It's very interesting to see how many other people with BAV also have PH.

 

[Karen]

I have a BAV and have been diagnose with pulmonary hypertension. I have mitral valve prolapse and insufficiecy too. It's very interesting to see how many other people with BAV also have PH.

Tammy, what do you do specifically to treat your PH? Is it secondary? And how you were diagnosed? Symptoms apart from the valve stuff?

 

[Karen]

Karen, I always, just called the other doctors myself for Justin. I was curious tho if you don't mind, do you see an adult congenital heart cardiologist or a for lack of a better word regular cardiologist? Lyn www.caringbridge.org/nj/justinw

Lyn, I see a "regular cardiologist."

 

[Nancy]

This is a newly approved inhalation med for Stage 3 or 4 PH. It's Ventavis

http://www.drugs.com/NDA/ventavis_040701.html

People on the PH website have had good relief with it.

 

[tammy70]

Karen, the pulmonary hypertension was found during one of my routine echo's. It is considered secondary PH. I have a diuretic that I can take when I am feeling short of breath and it will also help with the swelling in my feet and ankles.

 

[strawberry]

Karen, I don't mean to hijack YOUR thread either but I was curious about your chronic costochondritis. I see you had a coa repair in 1967. Does that involve a long incision along your ribs? Cause that is what I have from a PDA repair in 1968 and I have problems with Chondritis. In fact my ribs have been sore right at my sternum the last few days from playing in the pool with my kid. I wonder if there's any link between a surgery like that and costochondritis. I also have a rib that pops out of place (painfull) if I'm not careful. I'm also on the mild side with mild AI, TR and a myxomatous mitral valve. I checked my echo and my PA pressures aren't listed, does that mean they didn't check it, or just that it's ok? does anyone know?

 

[Karen]

Karen, I don't mean to hijack YOUR thread either but I was curious about your chronic costochondritis. I see you had a coa repair in 1967. Does that involve a long incision along your ribs? Cause that is what I have from a PDA repair in 1968 and I have problems with Chondritis. In fact my ribs have been sore right at my sternum the last few days from playing in the pool with my kid. I wonder if there's any link between a surgery like that and costochondritis. I also have a rib that pops out of place (painfull) if I'm not careful. I'm also on the mild side with mild AI, TR and a myxomatous mitral valve. I checked my echo and my PA pressures aren't listed, does that mean they didn't check it, or just that it's ok? does anyone know?

I can't say that my costochondritis has any connection to my coarct repair. That incision was along my back and under my left side, between the ribs. But the costochondritis stuff that I'm dealing with is in my upper chest -- feels more like lung or heart stuff than arthritis. (Which is one thing that keeps me coming back to the PH question...) But that's not to say that your problems could NOT be costochondritis. Good luck!

Karen

 

[Bunny_Rabbit]

Hi Karen

Hi Karen

I also have Secondary Pulmonary Hypertension, but due to Mirtal valve problems. From what I understood from my Cardiologist, as long as my valve is in check, then I don't have to worry about the PH.
But what does he know, he called me by the wrong name.

 

[Johnny Stephens]

I also have Secondary Pulmonary Hypertension, but due to Mirtal valve problems. From what I understood from my Cardiologist, as long as my valve is in check, then I don't have to worry about the PH.
But what does he know, he called me by the wrong name.

I would think that valve in check or not, if you still have PH, then you still need to be treated for it. (Unless it has actually resolved since your valve surgery.)

Since my surgery, everyone had "assumed" my PH had resolved because they couldn't see signs of it in my echoes. A right-heart cath performed for other reasons (Pacemaker) revealed this wasn't the case.

 

[tobagotwo]

Spvh

Spvh

There is a good likelihood that many people in the Waiting Room technically have secondary PH, as it is a natural consequence of the most common problems on this site. Fortunately, as Johnny Stephens points out, it usually dissipates when the valve issues are fixed. Unfortunately, as Johnny Stephens points out, it doesn't always happen that way.

The following is from a link Nancy provided. eMedicine, Pulmonary Hypertension by Davinder Jassal, MD, FRCPC (boldings mine):

Pulmonary venous hypertension is the most common form of pulmonary hypertension and usually due to left-sided heart disease. Pulmonary hypertension develops as a result of the obstruction of blood flow downstream from the pulmonary vein. Causes of pulmonary venous hypertension from distal to proximal of the pulmonary vasculature include coarctation of the aorta, aortic stenosis, aortic regurgitation, hypertrophic cardiomyopathy, constrictive pericarditis, restrictive cardiomyopathy, dilated cardiomyopathy, mitral stenosis, mitral regurgitation, and left atrial myxoma.

The cardiomyopathies weren't highlighted, but of course, a significant percentage of our membership lives with them as well.

So, as far as pre-op PH (which would usually be secondary pulmonary venous hypertension [SPVH]) it seems there should be some notice paid to it. Pre-VR, we should watch for it in echo reports, and ask the cardiologist if something should be done towards relieving it until the cause is repaired. Post-VR, we should watch to make sure it dissipates, and double-check to ensure that it's actually gone.

I'd like to think that usually, it will show up in an echo, or that there is at least some less invasive method of screening for it than a right-heart cath. Perhaps someone could elaborate on that.

I believe that asking the cardiologist to specifically have the tech look for that in the echo might help increase recognition of it. I think that the pulmonary number is frequently ignored, haphazardly calculated, or left off completely, as the focus of the echo is placed on the functioning of the new valves.

Of course, there are good techs and not-so-good techs, and sometimes the heart iteslf doesn't cooperate well in the views.

Best wishes,

 

[Nancy]

I couldn't agree more with Bob and Johnny. Assume NOTHING regarding PH. If it showed up in your echo ONCE, you should get copies of all further echoes and monitor it yourself, bearing in mind that echoes are not an accurate measure of PH, only a fuzzy snapshot.

Assuming that Joe's PH meant nothing, (on the part of his former cardiologist) because he had so many other issues, almost cost him his life. And it took a very long time to get him straightened out.

Again I'll say that the vast majority of cardiologists and pulmonologists may have never seen a patient with PH, and won't know how to proceed either in the diagnostic phase or in the treatment phase.

This is a disease you don't want to fool around with.

If your pressures are rising, get to a specialist and get a proper evaluation. Then you will know one way or the other whether you truly have PH.

You cannot determine yourself if it is primary or secondary. Even doctors who specilize disagree on that issue. I don't believe it matters in the early phases. It should be treated. Maybe you wouldn't need the heavier duty meds in the mild category, but there are other meds that can help relieve some of the symptoms.

Here is another link from a CHF website which is an excellent short resource on PH, symptoms diagnosis and treatment options.

http://www.chfpatients.com/ph.htm

 

[Karen]

now what?

now what?

Yesterday I sent a quick e-mail to the pulmonologist listed on the PH Association website. He's the chairman of the Pulmonary Division in a major hospital in Salt Lake City. He replied... He said that would hesitate to give much professional advice by email without the usual history, physical examination and laboratory data. And then, "Having said this, I think you should make an appointment to specifically review the results of your echocardiogram with your cardiologist. Then you can both decide whether further consultation is warranted." Needless to say, I am very curious as to what my cardio will say or do, if anything. I e-mailed him last night. But sometimes he takes a while to respond... I think I will be a little nervous if he does NOT think it's a good idea to go to the PH guy for further consultation. And yet I'm more than a little anxious about the possibility of having a right heart cath. 'Just waiting...

Nancy and Bob, thanks for those last two posts. It makes sense. I'm still not sure if I need to be doing anything, but all of this information has given me a lot to consider... It makes the most sense to "get rid" of whatever is causing PH. But valve replacement is a long way down the road yet.

A little MORE troublesome, is what you do AFTER surgery. Bob, I really do hope there's a happier solution to the riddle YOU are dealing with.

Thanks again for all the information you have shared.

Karen

 

[Karen]

response from my cardio

response from my cardio

My cardio responded to my e-mail... and encouraged me to come in so that we could discuss stuff. I did that today. He was not concerned with the numbers from the echo. It is his opinion that the echos may over-estimate pulmonary hypertension, but would rarely under-estimate it. He said we could do a right heart cath, and that maybe we should. But his final recommendation was that I take a little pill -- Lisionpril and Hydrochlorothiazide combination -- to address the high pulmonary pressure, and do another echo in 2 months OR a right heart cath. I don't know who or how it would be decided which is done...

However, I believe he prescribed that only to appease my anxiety. More important than the numbers in the echo, in his opinion, are symptoms of fatigue, decreased exercise tolerance, SOB. Also the progression. And there WAS some progression of PH as measured from the echo done last year compared to the one this year. That's the million dollar question for me. Ultimately, the "fix" is to replace the valve. But without several other factors in place, that is still a ways off. He DID say that the effects of the PH will dissipate after that -- no long-term damage -- at least not at the "mild" level that is indicated in the echo.

The issue is still clouded by my costochondritis diagnosis. Without question, some of my symptoms have responded to the medications I've taken for them (Vioxx and Voltaren), when I could take them. But even when I don't exercise or do the things that aggravate the costal stuff, I have pressure in my chest that is NOT "reproducible tenderness," nor does the pain respond to heat. A year ago, I walked/ran 3 miles every morning on my treadmill. As of May of this year, I do 2 miles and feel that that's all I've got. I don't have the crushing, squeezing pains in my chest that my PCP warns me to watch for. But my gut tells me that not all of the pressure I feel is arthritis in my chest... Can anyone help me out here? I have told my cardio repeatedly that I have no SOB, because I always look to the costochondritis explanation. But when the literature on costochondritis states that even doctors mistakenly presume heart or lung issues for costal chondritis, I wonder just how I AM to know for sure. This is my hope for this drug. IF the pressure I feel in my chest is due more to the valve or PH, the pill WILL make a difference, right? So maybe it's worth taking the pill just to test...

Does this sound like a reasonable course? The PA that talked to me for a long while before I saw the cardio said that of all the cardiologists in the valley (and virtually every cardiologist in the valley IS a part of this clinic) that my cardio and the one who read my last echo are the cardiologists to see for PH issues.

Lyn, you asked if my cardio was a regular cardio or an adult congenital heart cardiologist... I asked my cardio today about the studies mentioned here in the forum about the connection between coarctation of the aorta, bicuspid valves, and aortic aneurysms. I'm concerned that I may be at greater risk of having an ascending aortic aneurysm because of those other two conditions. (And yet the tests done to detect aneurysms are not routine, are they?) He said that HE is not an adult congenital heart cardiologist, but that he would talk about me to one who IS at the University of Utah next week, throwing into the mix the PH suggested by the echo. My cardio is also on staff at that hospital... Would it make sense to check out this other clinic for "children who managed to grow up to be adults"?

So sorry this is so long... I admit to feeling a little wiggy this week trying to be rational about this latest wrinkle, which may be nothing at all.

Bob, this is YOUR "fault." Had you not posed the questions that you did about your own probable PH, I'd probably still just be mulling this over on the back burner... But I think you're conclusions are right, and it kind of looks like a "waiting room" scenario. The unfortunate thing is, I was already there! 'Hope you don't end up there again.

Thanks for listening...
Karen

 

[Lynlw]

Karen, I would have the adult CHD doctor check you out, and since you have a few concerns about things now , it probably would be a good time. We have always been told that justin should always go to a cardiologist that specializes in CHDs since CHDs and the issues that go with them are so much different then what "regular" (sorry lol) cardiologist specialize in and see very often. Thankfully in the past few years, there are more ACHD centers, since more of the kids are making it to adulthood. I am glad your doctor was willing to talk to a doctor that specializes in chd since it shows he cares, Lyn www.caringbridge.org/nj/justinw