Abstract

Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality. BAV aortic dilatation is associated with an increased risk of adverse aortic events and represents a potentially lethal disease and hence a considerable medical burden. BAV with aortic dilatation warrants frequent monitoring, and elective surgical intervention is the only effective method to prevent dissection or rupture. The predictive value of the aortic diameter is known to be limited. The aortic diameter is presently still the main reference standard for surgical intervention owing to the lack of a comprehensive understanding of BAV aortopathy progression. This article provides a brief comprehensive review of the current knowledge on BAV aortopathy regarding clinical definitions, epidemiology, natural course, and pathophysiology, as well as hemodynamic and clinically significant aspects on the basis of the limited data available.

Highlights

  • Bicuspid aortic valve (BAV) disease is one of the most common congenital heart diseases, with a population incidence of about 0.5–2.0%, with male subjects the most commonly affected (Kong et al, 2017; Liu et al, 2018)

  • It is worth mentioning that Davies et al.’s (2007) study encouraged the exploration of prognostic differences in BAV patients with or without Coarctation of the aorta (CoA) but lacked the exploration of effects of risk factors of CoA severity and CoA repair history; this requires more research in the future

  • It should be noted that no single gene model can fully explain BAV or BAV aortopathy, meaning that BAV aortic disease might involve the genetic structure of very complex interactions among several genes

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Summary

Aortic Dilatation in Patients With Bicuspid Aortic Valve

Jing Wang , 1,2,3† Wenhui Deng1,2,3†, Qing Lv1,2,3, Yuman Li1,2,3, Tianshu Liu and Mingxing Xie1,2,3*. Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality. BAV aortic dilatation is associated with an increased risk of adverse aortic events and represents a potentially lethal disease and a considerable medical burden. BAV with aortic dilatation warrants frequent monitoring, and elective surgical intervention is the only effective method to prevent dissection or rupture. The predictive value of the aortic diameter is known to be limited. The aortic diameter is presently still the main reference standard for surgical intervention owing to the lack of a comprehensive understanding of BAV aortopathy progression. This article provides a brief comprehensive review of the current knowledge on BAV aortopathy regarding clinical definitions, epidemiology, natural course, and pathophysiology, as well as hemodynamic and clinically significant aspects on the basis of the limited data available

INTRODUCTION
Aortic Dilatation in BAV Patients
NATURAL COURSE
Genetic Predisposition
Histopathological Features of the Aorta
Imaging Diagnosis
Intervention and Prognosis
Special Populations
Findings
FUTURE PERSPECTIVE

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