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.
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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]
