scholarly journals Thoracic Aortic Aneurysm and Factors Affecting Aortic Dissection

2020 ◽  
Vol 10 (4) ◽  
pp. 153 ◽  
Author(s):  
Petr V. Chumachenko ◽  
Anton Yu. Postnov ◽  
Alexandra G. Ivanova ◽  
Olga I. Afanasieva ◽  
Maksim A. Afanasiev ◽  
...  
Keyword(s):  
Aortic Aneurysm ◽  
Aortic Dissection ◽  
Thoracic Aortic Aneurysm ◽  
Vasa Vasorum ◽  
Factors Affecting ◽  
Aneurysm Wall ◽  
Morphometric Investigation ◽  
Monocytes And Macrophages ◽  
Inflammatory Infiltrates ◽  
Von Willebrand

This study is aimed at investigating the relationship between inflammation, the number of vasa vasorum, and the presence of lipoprotein (a) [Lp(a)] in the aortic aneurysm wall, as well as the relationships of these pathological processes with the development of aneurysm wall dissection. To that end, we examined segments of aortic aneurysm wall, consisting of intima, media, and adventitia, collected from patients during aneurysm prosthetics intervention. The material was collected from 23 men and eight women aged from 33 to 69 years. Monoclonal antibodies to Lp(a), markers of monocytes and macrophages (CD68), T cells (CD3, CD4, and CD8), von Willebrand factor, endothelium NO synthase, and smooth muscle α-actin were used for morphological and morphometric investigation. The present study demonstrated that Lp(a) is not often found in biopsies of patients with thoracic aortic aneurysm. Morphological and morphometric investigation shows the connection of aortic dissection with the process of damage to its wall caused by inflammatory infiltrates, medianecroses, and the appearance of newly formed vasa vasorum in media.

Inflammatory infiltrates, vasa vasorum, and endothelial NO synthase in the wall of thoracic aortic aneurysm

2019 ◽  
Vol 81 (5) ◽  
pp. 45 ◽  
Author(s):  
P. V. Chumachenko ◽  
M. A. Afanasyev ◽  
A. G. Ivanova ◽  
I. P. Drobkova ◽  
G. I. Kheimets ◽  
...  
Keyword(s):  
Aortic Aneurysm ◽  
Thoracic Aortic Aneurysm ◽  
No Synthase ◽  
Vasa Vasorum ◽  
Endothelial No Synthase ◽  
Inflammatory Infiltrates

scholarly journals A post mortem assessment of a 25-year-old man with ascending aortic dissection and a novel MYLK variant

2015 ◽  
Vol 5 (1) ◽  
Author(s):  
Katelyn Hodge ◽  
Katherine G. Spoonamore ◽  
Christopher B. Griffith ◽  
David D. Weaver ◽  
Patricia B.S. Celestino-Soper ◽  
...  
Keyword(s):  
Genetic Testing ◽  
Aortic Aneurysm ◽  
Aortic Dissection ◽  
Thoracic Aortic Aneurysm ◽  
Aortic Rupture ◽  
Clinical Consequence ◽  
Post Mortem ◽  
Variant Of Uncertain Significance ◽  
Pathogenic Variants ◽  
Aortic Aneurysm And Dissection

We report on the process of <em>post mortem</em> evaluation and genetic testing following the death of a 25-year-old man due to ascending aortic dissection leading to aortic rupture. Following the negative clinical testing of a 12- gene thoracic aortic aneurysm and dissection panel, research testing revealed a novel c.5732A&gt;T (p.E1911V) variant in exon 34 of the MYLK gene (NM_053025). Two likely pathogenic variants in this gene have been reported previously in individuals with familial thoracic aortic aneurysm and dissection. Given the unclear clinical consequence of the variant found in our proband, we have classified this change as a variant of uncertain significance. In addition to discussing the complexity involved in variant interpretation, we recognize the need for additional research for more accurate <em>MYLK</em> interpretation. Finally, we comment on the unique challenges of <em>post mortem</em> genetic testing.

scholarly journals Familial Thoracic Aortic Aneurysm with Dissection Presenting as Flash Pulmonary Edema in a 26-Year-Old Man

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Sabry Omar ◽  
Tyler Moore ◽  
Drew Payne ◽  
Parastoo Momeni ◽  
Zachary Mulkey ◽  
...  
Keyword(s):  
Aortic Aneurysm ◽  
Aortic Dissection ◽  
Thoracic Aortic Aneurysm ◽  
Ascending Aorta ◽  
Screening Methods ◽  
Significant Past Medical History ◽  
Iliac Arteries ◽  
Aortic Aneurysm And Dissection ◽  
History Of ◽  
Chest X Ray

We are reporting a case of familial thoracic aortic aneurysm and dissection in a 26-year-old man with no significant past medical history and a family history of dissecting aortic aneurysm in his mother at the age of 40. The patient presented with cough, shortness of breath, and chest pain. Chest X-ray showed bilateral pulmonary infiltrates. CT scan of the chest showed a dissection of the ascending aorta. The patient underwent aortic dissection repair and three months later he returned to our hospital with new complaints of back pain. CT angiography showed a new aortic dissection extending from the left carotid artery through the bifurcation and into the iliac arteries. The patient underwent replacement of the aortic root, ascending aorta, total aortic arch, and aortic valve. The patient recovered well postoperatively. Genetic studies of the patient and his children revealed no mutations in ACTA2, TGFBR1, TGFBR2, TGFB2, MYH11, MYLK, SMAD3, or FBN1. This case report focuses on a patient with familial TAAD and discusses the associated genetic loci and available screening methods. It is important to recognize potential cases of familial TAAD and understand the available screening methods since early diagnosis allows appropriate management of risk factors and treatment when necessary.

Type A Aortic Dissection, Right-Sided Aortic Arch, and Thoracic Aortic Aneurysm

2009 ◽  
Vol 17 (6) ◽  
pp. 640-642 ◽  
Author(s):  
Daijiro Hori ◽  
Masashi Tanaka ◽  
Atsushi Yamaguchi ◽  
Hideo Adachi
Keyword(s):  
Aortic Aneurysm ◽  
Aortic Dissection ◽  
Aortic Arch ◽  
Thoracic Aortic Aneurysm ◽  
Type A ◽  
Type A Aortic Dissection

scholarly journals PersistentChlamydophila pneumoniaeinfection in thoracic aortic aneurysm and aortic dissection?

2010 ◽  
Vol 115 (3) ◽  
pp. 181-186 ◽  
Author(s):  
Marie Edvinsson ◽  
Stefan Thelin ◽  
Eva Hjelm ◽  
Göran Friman ◽  
Christina Nyström-Rosander
Keyword(s):  
Aortic Aneurysm ◽  
Aortic Dissection ◽  
Thoracic Aortic Aneurysm

scholarly journals Inhibition of HIPK2 Alleviates Thoracic Aortic Disease in Mice With Progressively Severe Marfan Syndrome

2021 ◽  
Author(s):  
Cristina I. Caescu ◽  
Jens Hansen ◽  
Brittany Crockett ◽  
Wenzhen Xiao ◽  
Pauline Arnaud ◽  
...  
Keyword(s):  
Protein Kinase ◽  
Aortic Aneurysm ◽  
Aortic Dissection ◽  
Marfan Syndrome ◽  
Thoracic Aortic Aneurysm ◽  
Acute Aortic Dissection ◽  
Transforming Growth Factor ◽  
Transforming Growth Factor Β ◽  
Aortic Disease ◽  
Computational Analyses

Objective: Despite considerable research, the goal of finding nonsurgical remedies against thoracic aortic aneurysm and acute aortic dissection remains elusive. We sought to identify a novel aortic protein kinase that can be pharmacologically targeted to mitigate aneurysmal disease in a well-established mouse model of early-onset progressively severe Marfan syndrome (MFS). Approach and Results: Computational analyses of transcriptomic data derived from the ascending aorta of MFS mice predicted a probable association between thoracic aortic aneurysm and acute aortic dissection development and the multifunctional, stress-activated HIPK2 (homeodomain-interacting protein kinase 2). Consistent with this prediction, Hipk2 gene inactivation significantly extended the survival of MFS mice by slowing aneurysm growth and delaying transmural rupture. HIPK2 also ranked among the top predicted protein kinases in computational analyses of genes differentially expressed in the dilated aorta of 3 MFS patients, which strengthened the clinical relevance of the experimental finding. Additional in silico analyses of the human and mouse data sets identified the TGF (transforming growth factor)-β/Smad3 signaling pathway as a potential target of HIPK2 in the MFS aorta. Chronic treatment of MFS mice with an allosteric inhibitor of HIPK2-mediated stimulation of Smad3 signaling validated this prediction by mitigating thoracic aortic aneurysm and acute aortic dissection pathology and partially improving aortic material stiffness. Conclusions: HIPK2 is a previously unrecognized determinant of aneurysmal disease and an attractive new target for antithoracic aortic aneurysm and acute aortic dissection multidrug therapy.

scholarly journals Fibromuscular dysplasia of aortic aneurysm vasa vasorum (a rare case report)

2020 ◽  
Vol 27 (4) ◽  
pp. 169-178
Author(s):  
S. S. Todorov ◽  
V. Yu. Deribas ◽  
A. S. Kaz’min ◽  
S. S. Todorov
Keyword(s):  
Aortic Aneurysm ◽  
Histological Examination ◽  
Fibromuscular Dysplasia ◽  
Aortic Wall ◽  
Vasa Vasorum ◽  
Wall Thickening ◽  
Elastic Fibres ◽  
Ascending Aortic Aneurysm ◽  
Aneurysm Wall ◽  
Smooth Myocytes

Aim. To describe a rare occurrence of fibromuscular vasa vasorum dysplasia of the aortic aneurysm wall.Materials and methods. Surgical material from the ascending aortic aneurysm wall was examined. Longitudinal strips of the aortic wall were excised for histological examination with subsequent 24-h fixation in 10% buffered formalin. A histological isopropanol assay was performed with an automated Logos microwave tissue processor (Milestone, Italy) with subsequent sample embedding into paraffin. Sections were obtained with a rotary microtome (Leica, Germany). Staining was performed with haematoxylin-eosin, van Gieson’s picrofuchsin, orcein for elastic fibres, Hotchkiss’ PAS reaction with alcian blue for glycosaminoglycans. Histological and histochemical properties of the aortic wall were studied and imaged with a Leica DM 1000 microscope (Germany) equipped with a camera ICC50 E at magnifications 40x, 100x, 200x, 400x.Results. The conducted histological examination of the aortic aneurysm wall revealed most pronounced changes in media and adventitia layers. Elastic fibres in media were swollen, homogeneous, crimped, with pronounced dystrophic and necrobiotic changes in smooth myocytes. Regions of compromised cells and elastic fibres in media contained pockets of alcian-positive glycosaminoglycans. Specific changes were revealed in adventitia vasa vasorum in the form of a pronounced wall thickening and lumen narrowing due to dysplastic fibromuscular tissues.Conclusion. A rare form of fibromuscular dysplasia of the vasa vasorum of the ascending aortic aneurysm wall observed in a 43 years-old woman demonstrated the morbid morphology of smooth myocytes, as well as fibrous collagenous and elastic structures. The described features were likely associated with the aortic wall trophic structure and aneurysm morphogenesis.

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