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Case Reports
. 2020 Jul 21;142(3):294-299.
doi: 10.1161/CIRCULATIONAHA.120.046892. Epub 2020 Jul 20.

Nosology Spectrum of the Bicuspid Aortic Valve Condition: Complex-Presentation Valvulo-Aortopathy

Hector I Michelena  1 , Saarwaani Vallabhajosyula  1 , Siddharth K Prakash  2
Affiliations
Case Reports

Nosology Spectrum of the Bicuspid Aortic Valve Condition: Complex-Presentation Valvulo-Aortopathy

Hector I Michelena et al. Circulation. .
No abstract available

Keywords: bicuspid aortic valve; congenital; heart valve diseases.

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Figures

Figure 1.
Figure 1.. Chest computed tomography angiography and brain magnetic resonance angiography.
A, Chest computed tomography angiogram demonstrates left ventricular (LV) outflow with dilated root (double-headed arrow) and proximal ascending aorta (Ao); 3-dimensional reconstruction shows root phenotype with enlarged sinuses in lateral view (B) and posterior view (C) with proximal ascending involvement. Note disproportional dilatation of noncoronary sinus (asterisk). The remainder of the distal thoracic aorta was normal. Magnetic resonance angiogram of the brain shows left supraclinoid intracranial aneurysm (arrows), originating from the left internal carotid artery (LIC), in lateral (D) and left anterior oblique (E) views.
Figure 2.
Figure 2.. Transthoracic echocardiography and intraoperative photographs.
A, Transthoracic echocardiography demonstrates significant dilatation of the aortic root in the left parasternal long-axis diastolic zoomed view (double-headed arrow; see Movie I in the Data Supplement. B, Systolic doming of the conjoined cusp (anterior cusp; arrow; see Movie I in the Data Supplement. C, High left parasternal view of the ascending aorta (Ao) showing progressive tapering of the aortic size from proximal to middle sections (double-headed arrows). D, Left parasternal short-axis diastolic view with zoom showing right (R)–left (L) fusion bicuspid aortic valve with dominant noncoronary cusp (N)/sinus (see Movie II in the Data Supplement. E, Intraoperative photograph with the root already excised shows the conjoined cusp (C) with prominent raphe (arrow). F, The excised bicuspid valve with a thickened conjoined cusp (C) and large, translucent noncoronary cusp (N). LA indicates left atrium; and RVOT, right ventricular outflow tract.
Figure 3.
Figure 3.. Nosologic spectrum of the bicuspid aortic valve (BAV) condition.
Complex-presentation and typical-presentation valvulo-aortopathies are described in the text and are risk factors for infective endocarditis and aortic dissection. The uncomplicated or undiagnosed category is more of a retrospective definition of cases of BAV in patients who never develop complications and remain undiagnosed through their lifetime, or are incidentally diagnosed by autopsy or imaging.
Figure 4.
Figure 4.. Genetic syndromes associated with bicuspid aortic valve (BAV) and clinical indications for genetic testing in patients with BAV.
See this image and copyright information in PMC

References

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