BAV does NOT mean a systemic connective tissue disease

[Wise]

"RESULTS We identified three morphologies: Type 1, fusion of right and left coronary cusp (N=152); Type 2, right and non-coronary fusion (N=39); and Type 3, left and non-coronary fusion (N=1). Comparing Type 1 and 2 BAV, there were no significant differences in age, height, weight, blood pressure, or aortic valve function. Type 1 was more common in men (69 vs. 45%,). The aortic sinuses were larger in Type 1, while Type 2 had larger arch dimensions. Myxomatous mitral valves were more common in Type 2 BAV (13% vs. 2.6%, p < 0.05). Three aortic shapes were defined: normal (N), sinus effacement (E), and ascending dilation (A). Comparing Type 1 to Type 2 BAV, shape N was more common in Type 1 (60% vs 32%), and Type A was more common in Type 2 (35% vs 54%,), Type E was rare (p<0.01 across all groups).

CONCLUSION A comprehensive BAV phenotype includes aortic shape. Type 1 BAV is associated with male gender and normal aortic shape but a larger sinus diameter. Type 2 leaflet morphology is associated with ascending aorta dilation, larger arch dimensions and higher prevalence of myxomatous mitral valve disease."

I don't really understand this. I wonder if, in laymen's terms, someone could tell if this addresses the connective tissue question? I'm the second in my family diagnosed with BAV. My aortic valve leaflets are cleanly split even halves, not fused. My brother had an aortic aneurysm form after he had a Ross Procedure. I suspect a connective tissue correlation.

 

[Susan BAV]

I only quoted the small abstract; perhaps there is more in the full text. I may be incorrect here Wise, but out of 191 bicuspid valve patients, they identified three categories (morphologies); but does the wording seem also to you to allow for more types, perhaps less common types? And I also echo your connective tissue question.

 

[PathFinder]

Right Oaktree

Right Oaktree

I already understood where was my mistake - in the title of the thread .

As I mentioned before, I looked in some medical dictionaries to check the definition of "systemic disorder". In abstract, it is a disorder, widened in the whole body, or a disorder, involving more than one organ, system or are.

If we define the BAV as the second definition above - YES, it is a systemic connective tissue disorder of a specific type. But I thought, I have already disscused it, by the description of the Fibrillin deficiency

Or I may be misunderstood many times, because my english is amateur, and I'm soory about that

Here I'd like to put another large study about the BAV in a large population, observed for 20 years.
***

Natural history of asymptomatic patients with normally functioning or minimally dysfunctional bicuspid aortic valve in the community.

Michelena HI, Desjardins VA, Avierinos JF, Russo A, Nkomo VT, Sundt TM, Pellikka PA, Tajik AJ, Enriquez-Sarano M.

Divisions of Cardiovascular Diseases, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.

BACKGROUND: Bicuspid aortic valve is frequent and is reported to cause numerous complications, but the clinical outcome of patients diagnosed with normal or mildly dysfunctional valve is undefined. METHODS AND RESULTS: In 212 asymptomatic community residents from Olmsted County, Minn (age, 32+/-20 years; 65% male), bicuspid aortic valve was diagnosed between 1980 and 1999 with ejection fraction > or =50% and aortic regurgitation or stenosis, absent or mild. Aortic valve degeneration at diagnosis was scored echocardiographically for calcification, thickening, and mobility reduction (0 to 3 each), with scores ranging from 0 to 9. At diagnosis, ejection fraction was 63+/-5% and left ventricular diameter was 48+/-9 mm. Survival 20 years after diagnosis was 90+/-3%, identical to the general population (P=0.72). Twenty years after diagnosis, heart failure, new cardiac symptoms, and cardiovascular medical events occurred in 7+/-2%, 26+/-4%, and 33+/-5%, respectively. Twenty years after diagnosis, aortic valve surgery, ascending aortic surgery, or any cardiovascular surgery was required in 24+/-4%, 5+/-2%, and 27+/-4% at a younger age than the general population (P<0.0001). No aortic dissection occurred. Thus, cardiovascular medical or surgical events occurred in 42+/-5% 20 years after diagnosis. Independent predictors of cardiovascular events were age > or =50 years (risk ratio, 3.0; 95% confidence interval, 1.5 to 5.7; P<0.01) and valve degeneration at diagnosis (risk ratio, 2.4; 95% confidence interval, 1.2 to 4.5; P=0.016; >70% events at 20 years). Baseline ascending aorta > or =40 mm independently predicted surgery for aorta dilatation (risk ratio, 10.8; 95% confidence interval, 1.8 to 77.3; P<0.01). CONCLUSIONS: In the community, asymptomatic patients with bicuspid aortic valve and no or minimal hemodynamic abnormality enjoy excellent long-term survival but incur frequent cardiovascular events, particularly with progressive valve dysfunction. Echocardiographic valve degeneration at diagnosis separates higher-risk patients who require regular assessment from lower-risk patients who require only episodic follow-up.

PMID: 18506017 [PubMed - indexed for MEDLINE]

 

[PathFinder]

yes, here is the data and the date

yes, here is the data and the date

Natural history of asymptomatic patients with normally functioning or minimally dysfunctional bicuspid aortic valve in the community.

Circulation. 2008 May 27;117(21):2776-84.

PMID: 18506017 [PubMed - indexed for MEDLINE]


Kind regards,
Ivo

 

[Susan BAV]

In terms of a bicuspid valve, no, not according to this way of classifying. There are three commissures in a normal tricuspid valve, a commissure between each of the three leaflets. (Edit: draw a "peace sign," and you will see what I mean.) Only one of the three commissures can be fused to make a bicuspid valve. Therefore there are only three types of bicuspid valves. The "Type" depends on which commissure is fused.

Now, it is true that there are quadricuspid and unicuspid valves, which are still different morphologies (that is to say, shapes or forms), and some authorities consider them to be the result of a similar tissue anomaly at the cellular level. But there can only be the three types of bicuspid valve, according to this particular system of classification.

I don't know but don't think these three would include Adam's surgeon's description.

 

[bird lady50]

i did not know that they two types

i did not know that they two types

I did not know that the was different types All I know that i have a bav. but which one i dont know . does it it make a diffrence then.

 

[Duff Man]

hey gang, I would really like to get a copy of the entire study mentioned by PathFinder here: http://heart.bmj.com/content/94/12/1634.abstract. I just came across some diagrams/pictures from this study on google images and they are very interesting.

I can't seem to download it without paying for it. It's 30 bucks through that site, but I don't see the entire article elsewhere on the net. Has PathFinder or anyone else been able to download it without paying? FWIW, I'm particularly interested in the full PDF version.