Patients with Bicuspid Aortopathy and Aortic Dilatation

Abstract

(1) Background: Bicuspid aortic valve (BAV) is the most frequent congenital cardiac disease. Alteration of ascending aorta diameter is a consequence of shear stress alterations due to haemodynamic abnormalities developed from inadequate valve cusp coaptation. (2) Objective: This narrative review aims to discuss anatomical, pathophysiological, genetical, ultrasound, and radiological aspects of BAV disease, focusing on BAV classification related to imaging patterns and flux models involved in the onset and developing vessel dilatation. (3) Methods: A comprehensive search strategy was implemented in PubMed from January to May 2022. English language articles were selected independently by two authors and screened according to the following criteria. (4) Key Contents and Findings: Ultrasound scan is the primary step in the diagnostic flowchart identifying structural and doppler patterns of the valve. Computed tomography determines aortic vessel dimensions according to the anatomo-pathology of the valve. Magnetic resonance identifies hemodynamic alterations. New classifications and surgical indications derive from these diagnostic features. Currently, indications correlate morphological results, dissection risk factors, and genetic alterations. Surgical options vary from aortic valve and aortic vessel substitution to aortic valve repair according to the morphology of the valve. In selected patients, transcatheter aortic valve replacement has an even more impact on the treatment choice. (5) Conclusions: Different imaging approaches are an essential part of BAV diagnosis. Morphological classifications influence the surgical outcome.

Aortic Valve Dysfunction and Aortopathy Based on the Presence of Raphe in Patients with Bicuspid Aortic Valve Disease

“…Aortopathy refers to the progressive dilatation of the ascending aorta and is defined as an indexed maximal aortic diameter exceeding 21 mm/m 2 of the body surface area (BSA) [ 11 ]. Ascending aortic dilation configurations were slightly modified from the Fazel classification, which was assigned to four types depending on whether the segment of the vessel was exclusively or predominantly involved in dilatation: normal aorta; type 1, isolated dilation of the ascending aorta root at the level of the sinus of Valsalva [ 12 ]; type 2, middle ascending dilatation at the level of the tubular ascending portion; and type 3, combined dilatation of the aortic root and mid-ascending aorta ( Figure 3 ) [ 12 ]. Aortic aneurysms are defined as those with dimensions greater than 50 mm.…”

Bicuspid aortic valve and its ascending aortopathy

“…The genetic theory of bicuspid aortopathy proposes that genetic abnormalities alter the development and function of the aortic valve and aorta, predisposing patients to BAV and aortic dilation. Within the last decade, research has identified candidate genes, transcription factor enzymes, metabolites, and signaling pathways implicated in abnormal aortic development and associated with aortic dilation [34][35][36][37].…”

Computed Tomography and Magnetic Resonance Imaging Findings of Bicuspid Aortic Valve and Related Abnormalities of the Heart and Thoracic Aorta

“…BAV-associated aortic dilatation can be classified into three types [ 25 ]. Type 1, the most common type, involves the dilatation of the aortic root, tubular ascending aorta, and proximal arch.…”