The bicuspid aortic valve: an integrated phenotypic classification of leaflet morphology and aortic root shape

Abstract

A comprehensive BAV phenotype includes aortic shape. Type 1 BAV is associated with male gender and normal aortic shape but a larger sinus diameter. Type 2 leaflet morphology is associated with ascending aorta dilatation , larger arch dimensions and higher prevalence of myxomatous mitral valve disease.

Novel Phenotypes in Bicuspid Aortic Valve Disease

“…Interestingly, Fazel et al (2008) found the highest prevalence of right coronary and noncoronary cusp fusion and the highest mean grade of aortic valve insufficiency in the cluster of BAV patients with dilatation of the tubular ascending aorta and aortic arch. These findings correlate appropriately with the data from the rheological and echocardiographic studies by Schaefer et al, (2008). Moreover, the retrospective study by Fazel et al, (2008) included only a selected (i.e., non-consecutive) cohort of BAV patients, for whom thoracic aortic imaging data were available for analysis.…”

“…This phenotypic classification has been proposed as a clinical and a research tool in order to precisely define BAV subgroups based on the combination of cusp morphology and proximal aortic shape. Another retrospective echocardiographic study demonstrated, analogously with Schaefer et al (2007Schaefer et al ( , 2008, that fusion of the right coronary and non-coronary cusps correlated with the more rapid growth of ascending aortic diameter in the pediatric population (Holmes et al, 2007). The pathogenetic background for clinical observation of the specific aortic shapes in BAV patients with different morphologic cusp fusion patterns has been elucidated in detailed in vivo rheological studies using sophisticated 4D magnetic resonance imaging .…”

“…The authors hypothesized that the differences in the spatial propagation of blood flow through the BAVs with fusion of the left coronary and right coronary cusps versus fusion of the right coronary and non-coronary cusps may lead to an inhomogeneous distribution of shear stress and, consequently, to differential alterations of the proximal aortic wall. The same authors advocated, in their subsequent retrospective analysis (Schaefer et al, 2008), an integrated phenotypic classification of BAV disease that includes both the cusp fusion pattern and the proximal aortic shape. Three different proximal aortic shapes in BAV disease were identified (Fig.…”

Current Topics on Bicuspid Aortic Valve: Clinical Aspects and Surgical Management

“…15) In an echocardiographic study of BAV by Schaefer and associates, the overall prevalence of myxomatous MV was 4.7% and varied according to the morphology of the aortic cups: its prevalence was 2.6% in the fused right and left cusp type and 13% in the fused right and non-coronary cusp type. 16) Roberts et al reported the combination of mitral valve prolapse and BAV, which is rare; however, a more detail examination to detect mitral valve dysfunction is recommended for surgical BAV patients. 17) Sievers and associates attempted to establish a classification system based on 5 years of data collected from the surgical specimens of 304 patients.…”

Bicuspid aortic valve disease

“…(24)(25)(26)(27)(28) Despite this challenge, the many prognostic this information is implicit in most current classifications. (27) In addition to valve morphology, more complete phenotypic classifications also include typing of aortic dilation.…”