Potential bicuspid valve issues...
Potential bicuspid valve issues...
There are a few relatively simultaneous threads here related to bicuspid valves. One is in regard to aneurysms
http://valvereplacement.com/forums/showthread.php?p=292565#post292565 which is undoubtedly something that many of us are concerned with, for ourselves and our families. Here are a couple of things I found related to the subject that I hope you will find informative and helpful:
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http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1464384 [published 2006]
Post-stenotic aortic dilatation
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Aortic dilatation occurring post AVR
Patients that undergo AVR often have some degree of ascending aortic dilatation. At present, replacing the aortic root at the time of AVR is controversial as the risk of aortic dilatation following AVR is uncertain. Some studies have been carried out to determine the natural history of ascending aortic dilatation following AVR.
Yasuda et al [4] carried out a retrospective analysis of patients (BAV n = 13, TAV n = 14), using echocardiography, before and after AVR and 18 BAV patients without AVR. Diameters were measured at the sinus of Valsalva, STJ and the proximal ascending aorta. The annual dilatation rate was calculated by dividing changes of diameter during the follow-up period by the BSA and the observational interval. Aortic dilatation in BAV patients was seen to be significantly faster than that of TAV at the proximal aorta only (0.18 compared to -0.08 mm/m2/year). There was no significant difference in the dilatation rates of BAV patients with and without AVR (0.03 and 0.02 mm/m2/year respectively). This study showed that AVR could not prevent progressive aortic dilatation in BAV. However, TAV patients did not show further aortic dilatation after AVR. It therefore seems that haemodynamic factors are not responsible for the ongoing dilatation in BAV...
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Conclusion
Post-stenotic aortic dilatation is most commonly seen in patients with a BAV. The degree of this dilatation, however, may not be related to the degree of AS and does not appear to be influenced by the occurrence of AVR. It is likely that the dilatation of the ascending aorta is due, mainly, to intrinsic pathology within the aortic wall rather than the haemodynamic effects of a dysfunctional AV.
There is controversy as to whether the ascending aorta should be replaced at the time of initial AV surgery, if the diameter of the aorta is < 5.5 cm. Most of the evidence suggests that the aorta will continue to dilate at an unknown rate. With improvements of surgical technique and perioperative management resulting in decreased morbidity and mortality following ARR, replacement of the ascending aorta should probably be considered for diameters of 4.5 ? 5.5 cm. Other therapeutic strategies being investigated that may reduce the rate of dilation are β-blockers, statins and the new synthetic MMP inhibitors.
Competing interests - The author(s) declare that they have no competing interests.
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http://www.expert-reviews.com/doi/abs/10.1586/14779072.3.2.295
Summary - Expert Review of Cardiovascular Therapy
March 2005, Vol. 3, No. 2, Pages 295-308 - (doi:10.1586/14779072.3.2.295)
Bicuspid aortic valve disease: recent insights in pathophysiology and treatment
Paul WM Fedak, Tirone E David, Michael Borger, Subodh Verma, Jagdish Butany and Richard D Weisel*
Bicuspid aortic valve is a common congenital cardiac malformation with a broad spectrum of clinical outcomes. Bicuspid aortic valve may go undetected throughout an individual?s lifetime or, alternatively, they may have devastating clinical consequences, resulting in death. Both clinicians and medical scientists have taken a renewed interest in the development, pathophysiology and treatment options for this subtle but often substantial clinical entity. Evidence is mounting to suggest that an underlying disease of the aorta is inherited with bicuspid aortic valve, although considerable controversy surrounds this theory. Novel molecular mechanisms underlying the valve and vascular pathologies, as well as new surgical therapies for these patients have been proposed in the past 10 years.
Cited by
Tamir Friedman, Arya Mani, John A Elefteriades. (2008) Bicuspid aortic valve: clinical approach and scientific review of a common clinical entity. Expert Review of Cardiovascular Therapy 6:2, 235-248
Online publication date: 1-Feb-2008.
Summary | Full Text | PDF (1095 KB) | PDF Plus (1171 KB)
JürgenScharhag, T.Meyer, I.Kindermann, G.Schneider, A.Urhausen, W.Kindermann. (2006) Bicuspid aortic valve. Clinical Research in Cardiology 95:4, 228-234
Online publication date: 1-May-2006.
CrossRef
Sing-Chien Yap, Johanna JM Takkenberg, Maarten Witsenburg, Folkert J Meijboom, Jolien W Roos-Hesselink. (2005) Aortic stenosis at young adult age. Expert Review of Cardiovascular Therapy 3:6, 1087-1098
Online publication date: 1-Nov-2005.
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Here's another one:
"The autosomal dominant inheritance of bicommissural aortic valve (BAV) (Online Mendelian Inheritance in Man #109730) in some families is well-documented; however, the inheritance of BAV with thoracic aortic aneurysm (TAA) is less clear. Whether the aneurysm is secondary to hemodynamic perturbation related to the valve abnormality or a primary manifestation of the disorder remains controversial. Guidelines are needed regarding the follow-up and treatment of these patients and their families. Thirteen families with at least one individual with TAA and BAV (BAV/TAA) were evaluated prospectively by standard echocardiographic methods or clinical history. Affected status was determined by the presence of BAV or TAA or a history of dissection, rupture, or surgical repair. Six of 13 families had at least two family members with both BAV and TAA, often in successive generations. Informatively, all 13 families had at least one family member with TAA in the absence of BAV. Thirty-five percent (39/110) of family members had BAV/TAA or TAA, and the majority of families (11/13) had maximal dilatation above the sinotubular junction (STJ). Vascular dissection or rupture occurred in seven of 13 families and in individuals with structurally normal aortic valves. Two families had non-manifesting, obligate carriers. Three families have members with other left heart outflow tract anomalies. This study confirms autosomal dominant inheritance with incomplete penetrance for BAV/TAA in these families. Furthermore, our data suggest that the component features, BAV and TAA, are independent manifestations of a single gene defect. To avoid the risk of early death, it is essential that all first-degree relatives receive echocardiographic follow-up at regular intervals regardless of the presence or absence of a BAV. This assessment must include imaging of the aortic region above the STJ."
http://www.ncbi.nlm.nih.gov/sites/en...ubmed_RVDocSum
