Bicuspid Aortic Valves: an Up-to-Date Review on Genetics, Natural History, and Management

Rashed, Eman; Dembar, Alexandra; Riasat, Maria; Zaidi, Ali N.

doi:10.1007/s11886-022-01716-2

“…As is known from their histories, most probably more than 50% of patients with BAV will undergo intervention for BAV (due to BAV stenosis or regurgitation) during their lifetime. Moreover, dilatation of the AA will require aortic surgery in more than 25% of these patients [15]. Cardiothoracic surgery in patients with ARVC may be associated with increased perioperative risk.…”

Section: Clinical Relevance Of Bav In Patients With Arvcmentioning

confidence: 99%

The bicuspid aortic valve and arrhythmogenic right ventricular cardiomyopathy. Unreported coexistence

Tyczyński¹,

Borowiec²,

Woźniak³

et al. 2023

Kardiol Pol

0

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“…However, AAA's biology of onset and progression is yet not well understood, and remains partially characterized, and particularly for the sporadic forms [2,3]. Such disease is also complicated in case of bicuspid aorta valve (BAV) anatomy [4][5][6]. AAA in BAV cases appears to be characterized by a typical pathogenesis and an early onset's age, as well as by an increased risk of evolving in severe aortic complications (i.e., dissection or rupture), when certain diameters, as well as a significant rate of calcification, are reached [4][5][6].…”

Section: Introductionmentioning

confidence: 99%

“…Such disease is also complicated in case of bicuspid aorta valve (BAV) anatomy [4][5][6]. AAA in BAV cases appears to be characterized by a typical pathogenesis and an early onset's age, as well as by an increased risk of evolving in severe aortic complications (i.e., dissection or rupture), when certain diameters, as well as a significant rate of calcification, are reached [4][5][6]. Currently, it is emerging the crucial role of endothelial dysfunction in AAA development and dissection/rupture associated with BAV, closely related to deregulation of regulatory pathways principally in endothelial cells (EC), and consequently in vascular smooth muscle cells (VSMC) [4][5][6].…”

Section: Introductionmentioning

confidence: 99%

“…AAA in BAV cases appears to be characterized by a typical pathogenesis and an early onset's age, as well as by an increased risk of evolving in severe aortic complications (i.e., dissection or rupture), when certain diameters, as well as a significant rate of calcification, are reached [4][5][6]. Currently, it is emerging the crucial role of endothelial dysfunction in AAA development and dissection/rupture associated with BAV, closely related to deregulation of regulatory pathways principally in endothelial cells (EC), and consequently in vascular smooth muscle cells (VSMC) [4][5][6]. Consistent with this, we have demonstrated in BAV cases, when compared with subjects with tricuspid 2 of 17 aortic valve (TAV), a deregulation of crucial EC pathways (i.e., Notch, TGF-β, TLR-4 and others), a reduced capacity of vascular repair significantly related to a decreased blood number of endothelial progenitor cells (EPC), and an altered T and B immune response to tissue damage [7][8][9][10][11].…”

Section: Introductionmentioning

confidence: 99%

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The Endothelial Transcription Factor ERG Mediates a Differential Role in the Aneurysmatic Ascending Aorta with Bicuspid or Tricuspid Aorta Valve: A Preliminary Study

Pisano

¹

,

Terriaca

²

,

Nardi

³

et al. 2022

IJMS

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The pathobiology of ascending aorta aneurysms (AAA) onset and progression is not well understood and only partially characterized. AAA are also complicated in case of bicuspid aorta valve (BAV) anatomy. There is emerging evidence about the crucial role of endothelium-related pathways, which show in AAA an altered expression and function. Here, we examined the involvement of ERG-related pathways in the differential progression of disease in aortic tissues from patients having a BAV or tricuspid aorta valve (TAV) with or without AAA. Our findings identified ERG as a novel endothelial-specific regulator of TGF-β-SMAD, Notch, and NO pathways, by modulating a differential fibrotic or calcified AAA progression in BAV and TAV aortas. We provided evidence that calcification is correlated to different ERG expression (as gene and protein), which appears to be under control of Notch signaling. The latter, when increased, associated with an early calcification in aortas with BAV valve and aneurysmatic, was demonstrated to favor the progression versus severe complications, i.e., dissection or rupture. In TAV aneurysmatic aortas, ERG appeared to modulate fibrosis. Therefore, we proposed that ERG may represent a sensitive tissue biomarker to monitor AAA progression and a target to develop therapeutic strategies and influence surgical procedures.

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“…AAA in BAV cases appears to be characterized by a typical pathogenesis and an early onset's age, as well as by an increased risk of evolving in severe aortic complications (i.e., dissection or rupture), when certain diameters, as well as a significant rate of calcification, are reached [4][5][6]. Currently, it is emerging the crucial role of endothelial dysfunction in AAA development and dissection/rupture associated with BAV, closely related to deregulation of regulatory pathways principally in endothelial cells (EC), and consequently in vascular smooth muscle cells (VSMC) [4][5][6]. Consistent with this, we have demonstrated in BAV cases, when compared with subjects with tricuspid aortic valve (TAV), a deregulation of crucial EC pathways (i.e., Notch, TGF-β, TLR-4 and others), a reduced capacity of vascular repair significantly related to a decreased blood number of endothelial progenitor cells (EPC), and an altered T and B immune response to tissue damage [7][8][9][10][11].…”

Section: Introductionmentioning

confidence: 99%

The Endothelial Transcription Factor ERG Mediates a Differential Role in the Aneurysmatic Ascending Aorta with Bicuspid or Tricuspid Aorta Valve: A Preliminary Study

Pisano¹,

Balistreri²,

Terriaca³

et al. 2022

Preprint

4

0

View full text Add to dashboard Cite

The pathobiology of ascending aorta aneurysms (AAA) onset and progression is not well understood and only partially characterized. AAA are also complicated in case of bicuspid aorta valve (BAV) anatomy. There is emerging evidence about the crucial role of endothelium-related pathways, which show in AAA an altered expression and function. Here, we examined the involvement of ERG-related pathways in the differential progression of disease in aortic tissues from patients having a BAV or tricuspid aorta valve (TAV) with or without AAA. Our findings identified ERG as a novel endothelial-specific regulator of TGF-β-SMAD, Notch, and NO pathways, by modulating a differential fibrotic or calcified AAA progression in BAV and TAV aortas. We provided evidence that calcification is correlated to different ERG expression (as gene and protein), which appears to be under control of Notch signaling. The latter, when increased, associated with an early calcification in aortas with BAV valve and aneurysmatic, was demonstrated to favor the progression versus severe complications, i.e., dissection or rupture. In TAV aneurysmatic aortas, ERG appeared to modulate fibrosis. Therefore, we proposed that ERG may represent a sensitive tissue biomarker to monitor AAA progression and a target to develop therapeutic strategies and influence surgical procedures.

show abstract

Bicuspid Aortic Valves: an Up-to-Date Review on Genetics, Natural History, and Management

Cited by 8 publications

References 66 publications

The bicuspid aortic valve and arrhythmogenic right ventricular cardiomyopathy. Unreported coexistence

The bicuspid aortic valve and arrhythmogenic right ventricular cardiomyopathy. Unreported coexistence

The Endothelial Transcription Factor ERG Mediates a Differential Role in the Aneurysmatic Ascending Aorta with Bicuspid or Tricuspid Aorta Valve: A Preliminary Study

The Endothelial Transcription Factor ERG Mediates a Differential Role in the Aneurysmatic Ascending Aorta with Bicuspid or Tricuspid Aorta Valve: A Preliminary Study

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