Aortic aneurysm: thoracic aortic aneurysm—diagnostic approach

ESC CardioMed ◽

10.1093/med/9780198784906.003.0607 ◽

2018 ◽

pp. 2570-2572

Author(s):

Oliver Gaemperli ◽

Udo Sechtem

Keyword(s):

Aortic Valve ◽

Aortic Aneurysm ◽

Aortic Aneurysms ◽

Diagnostic Approach ◽

Cross Sectional ◽

Safety Issues ◽

Thoracic Aortic Aneurysms ◽

Potential Safety ◽

Valve Function ◽

Choice Of Technique

The goals of the diagnostic approach to thoracic aortic aneurysms (TAAs) are to confirm the diagnosis, to measure diameter and define longitudinal extent, to determine involvement of the aortic valve and aortic branches, and to detect mural thrombus, dissection, and/or haematoma. A wide array of non-invasive and invasive imaging modalities is available to address all diagnostic challenges encountered in TAAs. Cross-sectional tomographic imaging methods are the preferred techniques for accurate measurement of TAA dimensions and extension, while echocardiography is crucial for assessing the aortic root and aortic valve function. The choice of technique should be based on patient factors and clinical scenario, but other factors such as operator skills, expertise and availability, costs, and potential safety issues of the techniques should be considered.

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The Value of Circulating Biomarkers in Bicuspid Aortic Valve-Associated Aortopathy

The Thoracic and Cardiovascular Surgeon ◽

10.1055/s-0036-1583525 ◽

2016 ◽

Vol 66 (04) ◽

pp. 278-286 ◽

Cited By ~ 7

Author(s):

Shiho Naito ◽

Mathias Hillebrand ◽

Alexander Bernhardt ◽

Annika Jagodzinski ◽

Lenard Conradi ◽

...

Keyword(s):

Aortic Valve ◽

Risk Stratification ◽

Bicuspid Aortic Valve ◽

Aortic Aneurysms ◽

Conventional Imaging ◽

Circulating Biomarkers ◽

Screening Process ◽

Cross Sectional ◽

Thoracic Aortic Aneurysms ◽

Definition Of

AbstractTraditional risk stratification model of bicuspid aortic valve (BAV) aortopathy is based on measurement of maximal cross-sectional aortic diameter, definition of proximal aortic shape, and aortic stiffness/elasticity parameters. However, conventional imaging-based criteria are unable to provide reliable information regarding the risk stratification in BAV aortopathy, especially considering the heterogeneous nature of BAV disease. Given those limitations of conventional imaging, there is a growing clinical interest to use circulating biomarkers in the screening process for thoracic aortic aneurysms as well as in the risk-assessment algorithms. We aimed to systematically review currently available biomarkers, which may be of value to predict the natural evolution of aortopathy in individuals with BAV.

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Quantitative not qualitative histology differentiates aneurysmal from nondilated ascending aortas and reveals a net gain of medial components

Scientific Reports ◽

10.1038/s41598-021-92659-1 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Sameh Yousef ◽

Nana Matsumoto ◽

Issam Dabe ◽

Makoto Mori ◽

Alden B. Landry ◽

...

Keyword(s):

Extracellular Matrix ◽

Quantitative Assessment ◽

Cell Number ◽

Aortic Aneurysms ◽

Aortic Dilatation ◽

Adaptive Responses ◽

Cross Sectional ◽

Thoracic Aortic Aneurysms ◽

Medial Degeneration ◽

The Media

AbstractMedial degeneration is a common histopathological finding in aortopathy and is considered a mechanism for dilatation. We investigated if medial degeneration is specific for sporadic thoracic aortic aneurysms versus nondilated aortas. Specimens were graded by pathologists, blinded to the clinical diagnosis, according to consensus histopathological criteria. The extent of medial degeneration by qualitative (semi-quantitative) assessment was not specific for aneurysmal compared to nondilated aortas. In contrast, blinded quantitative assessment of elastin amount and medial cell number distinguished aortic aneurysms and referent specimens, albeit with marked overlap in results. Specifically, the medial fraction of elastin decreased from dilution rather than loss of protein as cross-sectional amount was maintained while the cross-sectional number, though not density, of smooth muscle cells increased in proportion to expansion of the media. Furthermore, elastic lamellae did not thin and interlamellar distance did not diminish as expected for lumen dilatation, implying a net gain of lamellar elastin and intralamellar cells or extracellular matrix during aneurysmal wall remodeling. These findings support the concepts that: (1) medial degeneration need not induce aortic aneurysms, (2) adaptive responses to altered mechanical stresses increase medial tissue, and (3) greater turnover, not loss, of mural cells and extracellular matrix associates with aortic dilatation.

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Abstract 15169: De Novo Variants of USP10 in Early Onset Bicuspid Aortic Valve Disease

Circulation ◽

10.1161/circ.142.suppl_3.15169 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Siddharth K Prakash ◽

Angela T Yetman ◽

Hector I Michelena ◽

Malenka M Bissell ◽

Yuli Y Kim ◽

...

Keyword(s):

Aortic Valve ◽

Bicuspid Aortic Valve ◽

Early Onset ◽

Rare Variants ◽

De Novo ◽

Late Onset ◽

Copy Number Variants ◽

Aortic Aneurysms ◽

Thoracic Aortic Aneurysms ◽

Aortic Dissections

Introduction: Bicuspid Aortic Valve (BAV), the most common congenital heart defect, is a major cause of aortic regurgitation or stenosis requiring valve replacement and thoracic aortic aneurysms predisposing to acute aortic dissections (TAD). The spectrum of BAV ranges from severe early onset valve and aortic complications to sporadic late onset disease. Hypothesis: Early onset BAV (EBAV) cases with valve or aortic complications that require intervention prior to age 30 are enriched for rare genetic variants that cause BAV and TAD. Methods: We performed whole exome sequencing of 147 EBAV cases in 141 families who were enrolled in the UTHealth Bicuspid Aortic Valve Research Registry. Candidate variants in the EBAV cohort (26% female, mean age 18, 44% with TAD) were compared to unselected controls from the Genome Aggregation Database (gnoMAD) and the Database of Genotypes and Phenotypes (dbGAP). We considered variants with minor allele frequencies (MAF) < 1%, Combined Annotation Dependent Depletion (CADD) scores > 25, and damaging (Polyphen-2) or deleterious (SIFT) functional prediction scores. Genomic copy number variants (CNVs) were detected using CoNIFER and prioritized when deletions involved genes with probability of loss intolerance (pLI) > 0.9. Variants were validated using quantitative PCR or Sanger sequencing. Results: We identified 6 rare variants of USP10 in 6 EBAV families (4% of cohort): 4 CNVs (2 duplications and 2 deletions) that are rare in dbGAP controls (4 in 15,414) and 2 deleterious rare missense variants (MAF<5x10 -5 in gnoMAD). Two of the 4 CNVs were de novo events in trios. In contrast, rare deleterious variants of the known causal BAV genes NOTCH1 (1), ROBO4 (1), GATA4 (1), GATA5 (1), and SMAD6 (4) were found in 7 total families. USP10 encodes a ubiquitin peptidase that is required for endothelial Notch signaling during vascular development. Conclusions: We identified rare and de novo variants of USP10 that implicate USP10 as a new candidate gene for BAV.

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Thoracic Aortic Aneurysm Repair with Endovascular Stent-Grafts

Vascular Medicine ◽

10.1177/1358863x9700200205 ◽

1997 ◽

Vol 2 (2) ◽

pp. 98-103 ◽

Cited By ~ 32

Author(s):

Charles P Semba ◽

R Scott Mitchell ◽

D Craig Miller ◽

Noriyuki Kato ◽

Stephen T Kee ◽

...

Keyword(s):

High Risk ◽

Aortic Aneurysm ◽

Stent Graft ◽

Aortic Aneurysms ◽

Stent Grafts ◽

Thoracic Aortic Aneurysms ◽

Risk Patients ◽

Aneurysm Repair ◽

Aortic Aneurysm Repair ◽

Endoluminal Stent

The purpose of the study was to describe the clinical experience in using endoluminal stent-grafts for the treatment of thoracic aortic aneurysms in high-risk patients. Patients with aneurysms of the descending thoracic aorta who were considered high surgical risks underwent evaluation for endoluminal repair. The prosthesis was constructed from Z stents covered with polyester fabric using dimensions based upon preprocedural computed tomography scans and angiography. Through a femoral arteriotomy or left retroperitoneal flank incision, a 22–24 Fr delivery catheter was inserted and advanced through the aorta to the target site under fluoroscopic guidance in the operating suite. The stent-graft prosthesis was deployed at the site of the aneurysm. 44 patients (36 male, 8 female; mean age 36 years) underwent stent-graft repair for thoracic aneurysms (mean diameter 6.3 cm). The deployment was technically successful in all cases, with complete aneurysm thrombosis in 88%. The 30-day perioperative mortality rate was 6.8% and 35-month actuarial survival was 82%. There were no cases of stent migration, surgical conversion or intraprocedural death. Paraplegia occurred in two patients who underwent simultaneous surgical infrarenal aortic aneurysm repair immediately followed by stent-graft placement for a coexisting thoracic aneurysm. The conclusion was that placement of endoluminal stent-grafts for repair of thoracic aortic aneurysms is technically feasible in high-risk patients in whom conventional surgery is contraindicated. Long-term studies are needed to determine protection against aneurysm rupture and patient survival.

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Biomechanical Analysis of an Aortic Aneurysm Model and Its Clinical Application to Thoracic Aortic Aneurysms for Defining “Saccular” Aneurysms

Journal of the American Heart Association ◽

10.1161/jaha.114.001547 ◽

2015 ◽

Vol 4 (1) ◽

Cited By ~ 6

Author(s):

Takafumi Akai ◽

Katsuyuki Hoshina ◽

Sota Yamamoto ◽

Hiroaki Takeuchi ◽

Youkou Nemoto ◽

...

Keyword(s):

Aortic Aneurysm ◽

Clinical Application ◽

Aortic Aneurysms ◽

Biomechanical Analysis ◽

Thoracic Aortic Aneurysms ◽

Aneurysm Model ◽

Saccular Aneurysms

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Atlas-Based Evaluation of Hemodynamic in Ascending Thoracic Aortic Aneurysms

Applied Sciences ◽

10.3390/app12010394 ◽

2021 ◽

Vol 12 (1) ◽

pp. 394

Author(s):

Chiara Catalano ◽

Valentina Agnese ◽

Giovanni Gentile ◽

Giuseppe M. Raffa ◽

Michele Pilato ◽

...

Keyword(s):

Aortic Valve ◽

Fluid Shear Stress ◽

Aortic Aneurysms ◽

Disease Assessment ◽

Complex Nature ◽

Thoracic Aortic Aneurysms ◽

Statistical Shape ◽

Specific Shape ◽

Mode 3 ◽

Aortic Size

Atlas-based analyses of patients with cardiovascular diseases have recently been explored to understand the mechanistic link between shape and pathophysiology. The construction of probabilistic atlases is based on statistical shape modeling (SSM) to assess key anatomic features for a given patient population. Such an approach is relevant to study the complex nature of the ascending thoracic aortic aneurysm (ATAA) as characterized by different patterns of aortic shapes and valve phenotypes. This study was carried out to develop an SSM of the dilated aorta with both bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV), and then assess the computational hemodynamic of virtual models obtained by the deformation of the mean template for specific shape boundaries (i.e., ±1.5 standard deviation, σ). Simulations demonstrated remarkable changes in the velocity streamlines, blood pressure, and fluid shear stress with the principal shape modes such as the aortic size (Mode 1), vessel tortuosity (Mode 2), and aortic valve morphologies (Mode 3). The atlas-based disease assessment can represent a powerful tool to reveal important insights on ATAA-derived hemodynamic, especially for aneurysms which are considered to have borderline anatomies, and thus challenging decision-making. The utilization of SSMs for creating probabilistic patient cohorts can facilitate the understanding of the heterogenous nature of the dilated ascending aorta.

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