Living with a Bicuspid Aoritc Valve

[della_anne]

Mark,


I was also born with a bicuspid Aortic valve, although the doctors found out it about it right after I was born. The cardiologist told me that eventually it would have to be replaced, but in the meantime it was working well and didn't need anything done to it right away. I lived with the bicuspid Aortic valve throughout my childhood until I was 12 years old. The doctor never mentioned anything about Aortic Anuresym or Aortic Dissection.The rest of the story might scare you so I don't want to go into too many details. When I was 12 bacteria infected my heart, I got a porcine valve to replace theAortic valve. And then later the porcine valve was replaced with a mechanical valve.

 

[BAV Pt's daughter]

You're in the right place

You're in the right place

Hello Mark!!
I can tell you that you've come to the right place. There are wonderful, and very knowledgeable people--friends--here.
My mother was diagnosed with BAVD in her early years and just had AVR with aortic aneurysm repair in October at Cedars-Sinai by Dr Raissi... what a wonderful place, and even more wonderful doctor. We flew all the way from the east coast to LA for him and that facility. While there, Dr Raissi had his own suspicions about the "essentially normal" echo of my own and sent me on my way for an MRI, only to find out that I too have BAVD, mild aortic insufficiency, but the valve functions again "essentially normally" for now. They never clued in to it on the echo... For now, my BP is actually too low, b/c he'd like me to get something to lower my heart rate (resting HR is usually >110), but the low BP won't tolerate it for now. Only other thing is weight lifting restrictions that are more significant than I'd like and await some more fancy "3T" MRI to come out to the public.
Coming to terms was a little easier for me with going through it with mom, but then again scarier at times, as I have always been so healthy.
But this site is full of people that have been there, done that, and are full of information, strength, and support. I never would've made it through that stressful time of mom's surgery without these guys. Even got to meet a couple of them while out in LA. Best of luck, and do follow up regularly, please, MUCH more regularly than every 10 years. My mom's ascending aorta went from normal to "time for surgical intervention" in a year, so you can't be too careful. Besides, wouldn't you rather be too careful than not careful enough?
Keep everyone updated!!

 

[marka]

From what I gathered if you have a Bicuspid Aortic Valve you have Bicuspid Aoritc Valve Disease, the connective tissue disorder in the Aorta just varies from person to person?

From my experiences with Cardio's I feel they do not look at the BAV as a serious problem (probably because they see a lot worse) and there should be more focus on the Aorta, more so than the BAV. But I'm no Doctor and could be totally wrong, it's just my impressions so far.

Thanks

 

[PJmomrunner]

For ease of reference I have quoted the disease overview from the Bicuspid Aortic Foundation website below. Their overview doesn't make a point of distinquishing between a BAV and BAVD, but my own interpretation is that it doesn't preclude BAV existing apart from BAVD. Possibly this is something I've arrived at on my own (in my many hours spent poring over studies my doctors and family, I suspect, wish I wouldn't ), but I believe that BAV's do exist apart from BAVD. T

hat said, I think a congenital BAV (meaning one that was genetically predestined, whether handed down in an autosomal dominant manner or by spontaneous mutation) is accompanied by suspect ascending aortic tissue and an ascending aorta that is, on average, larger than normal, but not necessarily aneurysmal.(http://www.ncbi.nlm.nih.gov/entrez/..._uids=12842243&query_hl=6&itool=pubmed_docsum)


Several people on this website have stated that their surgeons, upon excising their BAV, have declared that their valve leaflets became fused, presumably by disease (endocarditis, rheumatic fever...) and the study proclaiming 89% heritability (http://www.ncbi.nlm.nih.gov/entrez/...ids=15234422&query_hl=64&itool=pubmed_docsum), at least, leaves room for other origins. I happened to be researching (okay, obsessing) today about another lovely condition (I happen to have) called Antiphospholipid Syndrome (APS) and found that it too can result in fused, thickened, always regurgitant (never stenotic) aortic valve leaflets which are bicuspid.

So, my take (FWIW) is that BAV usually is the same as BAVD, but not always.

BTW, if anyone else has APS, I'd love to hear about your experiences...perhaps I'll start a thread....

What is Bicuspid Aortic Valve Disease (BAVD)?


Overview

Bicuspid aortic valve disease is more than a bicuspid aortic valve.

This condition gets its name from the bicuspid aortic valve, which is the most common congenital abnormality of the heart. The bicuspid aortic valve is an important marker of this disease and might be what is first noticed. However, more than the aortic valve is involved. To indicate this, sometimes the term bicuspid aortic disease is used.

Current clinical and scientific studies are revealing that bicuspid aortic valve disease is not a simple valve condition. It increasingly appears to be a genetically based connective tissue disorder. An inclusive definition, listing the major areas of the body possibly affected, is needed. Not everything is understood today, but enough is known to identify several major characteristics of this condition.

BAVD includes the following:

? Bicuspid aortic valve
? Thoracic aortic dilation, aneurysm, or dissection
? Labile hypertension (high blood pressure which changes quickly with stress or exercise)

It may also include:

? Mitral valve leakage
? Coarctation of the aorta
? Kidney or liver cysts
? Near-sighted vision
? Musculoskeletal degenerative changes (scoliosis, degenerative spinal discs, flexible joints, flat feet, etc.)
? Aneurysm of smaller arteries, including head vessels (berry aneurysms)

Not everyone has all of the listed characteristics. However, those diagnosed with a bicuspid aortic valve, who may have wondered why they also have high blood pressure, a leaking mitral valve, or poor eye sight, will recognize themselves in the definition above.

BAVD does not simply mean that some day, when you are older, you may need aortic valve surgery.

In many people, BAVD is not just an abnormally shaped aortic valve that may some day, when they are older, need to be repaired or replaced. A broader description is needed to include those who:
? require aortic valve treatment in infancy, childhood, or as young adults.
? develop an aortic aneurysm.
? develop an aortic aneurysm after their bicuspid aortic valves have been replaced.
? experience dissection of their ascending aorta.
? have mitral valve failure.

The challenge for those who have BAVD is to understand what it means to them individually.

BAVD is different in different people, but there are some patterns.

Bicuspid aortic valve disease is not a uniform condition. It is different in different people. Among those with bicuspid aortic valve disease there are some sub-groups that are beginning to emerge.

? One group includes those who are younger, (in their 20's or 30's), and have aortic valve insufficiency (leakage) and an ascending aortic aneurysm. It appears that this group has more fragile tissue in the heart valves, aorta, and possibly other arteries.

? Another group are those, ranging in age from about 40 to 60, who develop aortic valve calcification and stenosis (narrowing).

? Many develop consistently high blood pressure or high blood pressure that rises and falls quickly (labile hypertension), while a very few have normal blood pressure.

? When more studies and research are done, it should be possible to sort out all of the variations of bicuspid aortic disease.

Not everything is understood, but the most dangerous side of BAVD is known.

While not everything is understood about bicuspid aortic disease, the most dangerous aspects of it are known. This disease presents the greatest threat to life due to abnormalities that are seen in the heart valves and the aorta.

? As with any heart valve condition, it is important to obtain expert medical care for a bicuspid aortic valve. However, focusing on the aortic valve alone is not enough.

? Ignoring other potential affects of this disease can be dangerous. Aortic aneurysm and dissection are serious, life-threatening conditions.

? It is not safe to think that if the bicuspid aortic valve has been removed, repaired, or continues to work well that everything is all right, because there is still a risk of aortic aneurysm and/or dissection

.

 

[Gemma]

For ease of reference I have quoted the disease overview from the Bicuspid Aortic Foundation website below. Their overview doesn't make a point of distinquishing between a BAV and BAVD, but my own interpretation is that it doesn't preclude BAV existing apart from BAVD. Possibly this is something I've arrived at on my own (in my many hours spent poring over studies my doctors and family, I suspect, wish I wouldn't ), but I believe that BAV's do exist apart from BAVD.

That was my interpretation too. Jim was one of those with a "fused" valve, but I wonder if it was more that the 3 leaflets never separated in the first place? Basically 2 were half-joined and the 3rd wasn't big enough for the gap. Anyway, no mention has ever been made of Jim having any aorta problems.

I'm quite happy to believe that Jim's doctors/surgeon would have spotted and informed us of any problems aside from the BAV. He doesn't have any of the other symptoms of BAVD, so I figure why go looking for problems? Maybe my optimism is delusional, but I like to think things are going to be OK from now on . I'm more than happy to stick with the "BAV's do exist apart from BAVD" theory.

 

[marka]

I came across some information that basically says dissections can occur regardless of Aorta size. Maybe I'm reading it wrong but the table lists a higher risk between 3.5cm and 4.0 cm, why would this be? The link to the document is below, maybe someone can clear this up for me? I'm confused.

http://www.jsvs.org/ja/journal/meeting/lecture.php?mc=32&no=5

Thanks
Mark