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

2021 ◽  
Vol 8 (12) ◽  
pp. 174
Author(s):  
Nan Zhou ◽  
Qianhao Zhao ◽  
Rui Li ◽  
Da Zheng ◽  
Yuxi Xiao ◽  
...  

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.

2016 ◽  
Vol 2016 ◽  
pp. 1-10 ◽  
Author(s):  
Betti Giusti ◽  
Stefano Nistri ◽  
Elena Sticchi ◽  
Rosina De Cario ◽  
Rosanna Abbate ◽  
...  

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.


Circulation ◽  
2005 ◽  
Vol 112 (2) ◽  
pp. 200-206 ◽  
Author(s):  
Philippe Khau Van Kien ◽  
Flavie Mathieu ◽  
Limin Zhu ◽  
Alain Lalande ◽  
Christine Betard ◽  
...  

2020 ◽  
Vol 34 (5) ◽  
pp. 641-650 ◽  
Author(s):  
Fang Wang ◽  
Yimin Tu ◽  
Yanxiang Gao ◽  
Houzao Chen ◽  
Jinjing Liu ◽  
...  

2004 ◽  
Vol 12 (3) ◽  
pp. 173-180 ◽  
Author(s):  
Philippe Khau Van Kien ◽  
Jean-Eric Wolf ◽  
Flavie Mathieu ◽  
Limin Zhu ◽  
Nicolas Salve ◽  
...  

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