nnResearch progress on B cells and thoracic aortic aneurysm/dissection

Bicuspid valve disease is associated with the development of thoracic aortic aneurysm. The molecular mechanisms underlying this association still need to be clarified. Here, we evaluated the circulating levels of T and B lymphocyte subsets associated with the development of vascular diseases in patients with bicuspid aortic valve or tricuspid aortic valve with and without thoracic aortic aneurysm. We unveiled that the circulating levels of the MAIT, CD4+IL−17A+, and NKT T cell subsets were significantly reduced in bicuspid valve disease cases, when compared to tricuspid aortic valve cases in either the presence or the absence of thoracic aortic aneurysm. Among patients with tricuspid aortic valve, these cells were higher in those also affected by thoracic aortic aneurysm. Similar data were obtained by examining CD19+ B cells, naïve B cells (IgD+CD27−), memory unswitched B cells (IgD+CD27+), memory switched B cells (IgD−CD27+), and double-negative B cells (DN) (IgD−CD27−). These cells resulted to be lower in subjects with bicuspid valve disease with respect to patients with tricuspid aortic valve. In whole, our data indicate that patients with bicuspid valve disease show a quantitative reduction of T and B lymphocyte cell subsets. Future studies are encouraged to understand the molecular mechanisms underlying this observation and its pathophysiological significance.

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Is Emilin-1 a molecular link contributing to the extension of thoracic aortic aneurysm dissection and increasing the magnitude of the associated hypertension

Integrative Molecular Medicine ◽

10.15761/imm.1000254 ◽

2016 ◽

Vol 3 (6) ◽

Author(s):

Simon W Rabkin

Keyword(s):

Aortic Aneurysm ◽

Thoracic Aortic Aneurysm ◽

Aneurysm Dissection

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A Case Based Approach to Clinical Genetics of Thoracic Aortic Aneurysm/Dissection

BioMed Research International ◽

10.1155/2016/9579654 ◽

2016 ◽

Vol 2016 ◽

pp. 1-10 ◽

Cited By ~ 5

Author(s):

Betti Giusti ◽

Stefano Nistri ◽

Elena Sticchi ◽

Rosina De Cario ◽

Rosanna Abbate ◽

...

Keyword(s):

Aortic Aneurysm ◽

Thoracic Aortic Aneurysm ◽

State Of The Art ◽

Genetic Profile ◽

Clinical Genetics ◽

Clinical Genetic ◽

Increased Risk ◽

Rising Incidence ◽

Aneurysm Dissection

Thoracic aortic aneurysm/dissection (TAAD) is a potential lethal condition with a rising incidence. This condition may occur sporadically; nevertheless, it displays familial clustering in >20% of the cases. Family history confers a six- to twentyfold increased risk of TAAD and has to be considered in the identification and evaluation of patients needing an adequate clinical follow-up. Familial TAAD recognizes a number of potential etiologies with a significant genetic heterogeneity, in either syndromic or nonsyndromic forms of the manifestation. The clinical impact and the management of patients with TAAD differ according to the syndromic and nonsyndromic forms of the manifestation. The clinical management of TAAD patients varies, depending on the different forms. Starting from the description of patient history, in this paper, we summarized the state of the art concerning assessment of clinical/genetic profile and therapeutic management of TAAD patients.

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Mapping of Familial Thoracic Aortic Aneurysm/Dissection With Patent Ductus Arteriosus to 16p12.2–p13.13

Circulation ◽

10.1161/circulationaha.104.506345 ◽

2005 ◽

Vol 112 (2) ◽

pp. 200-206 ◽

Cited By ~ 51

Author(s):

Philippe Khau Van Kien ◽

Flavie Mathieu ◽

Limin Zhu ◽

Alain Lalande ◽

Christine Betard ◽

...

Keyword(s):

Aortic Aneurysm ◽

Patent Ductus Arteriosus ◽

Thoracic Aortic Aneurysm ◽

Ductus Arteriosus ◽

Aneurysm Dissection ◽

Patent Ductus

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Does Anatomic Phenotype of Mitral Annular Disjunction Impact Survival? An Autopsy-Based Retrospective Study

Journal of Cardiovascular Development and Disease ◽

10.3390/jcdd8120174 ◽

2021 ◽

Vol 8 (12) ◽

pp. 174

Author(s):

Nan Zhou ◽

Qianhao Zhao ◽

Rui Li ◽

Da Zheng ◽

Yuxi Xiao ◽

...

Keyword(s):

Retrospective Study ◽

Cardiovascular Diseases ◽

Aortic Aneurysm ◽

Thoracic Aortic Aneurysm ◽

Mitral Annulus ◽

Age At Death ◽

All Cause Mortality ◽

Clinically Significant ◽

Aneurysm Dissection ◽

Additive Influence

Controversies have been raised regarding the prevalence and potential clinical significance of mitral annular disjunction (MAD). We aim to address the anatomic characteristics of MAD and their association, if any, on survival. We retrospectively reviewed 1373 consecutive dissected hearts (1017 men, mean age at death 44.9 ± 0.4 y) and frequently detected MAD (median disjunctional length: 2.0 mm, range: 1.5 mm~8.5 mm), with the prevalence of 92.1% over the entire mitral annulus and 74.9% within the posterior annulus (pMAD). The presence of pMAD was associated with increased all-cause mortality (45 y vs. 49 y, hazard ratio [HR]: 1.28, 95% confidence interval [CI]: 1.11~1.47, p < 0.001), which persisted in the context of cardiovascular diseases (CVDs; 46 y vs. 51 y, HR: 1.33, 95% CI: 1.14~1.56, p < 0.001) but was insignificant in those without CVDs. Compared to those without pMAD, individuals with pMAD affecting the entire posterior annulus or having a mean standardized length of ≥1.78 showed other clinically significant cardiovascular phenotypes, including the enlargement of aortic annular circumferences and a higher occurrence of thoracic aortic aneurysm/dissection. This largest series of autopsies show that MAD is a common phenotype that may exert additive influence on the survival of individuals. It is necessary to establish a precise classification and stratification of MAD.

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