scholarly journals Rare Causes of Arterial Hypertension and Thoracic Aortic Aneurysms—A Case-Based Review

Diagnostics ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 446
Author(s):  
Svetlana Encica ◽  
Adrian Molnar ◽  
Simona Manole ◽  
Teodora Filan ◽  
Simona Oprița ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Aortic Aneurysm ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Gene Mutations ◽  
Young Male ◽  
Renin Angiotensin System ◽  
Aortic Aneurysms ◽  
Thoracic Aortic Aneurysms ◽  
Medial Degeneration

Thoracic aortic aneurysms may result in dissection with fatal consequences if undetected. A young male patient with no relevant familial history, after having been investigated for hypertension, was diagnosed with an ascending aortic aneurysm involving the aortic root and the proximal tubular segment, associated with a septal atrial defect. The patient underwent a Bentall surgery protocol without complications. Clinical examination revealed dorso–lumbar scoliosis and no other signs of underlying connective tissue disease. Microscopic examination revealed strikingly severe medial degeneration of the aorta, with areas of deep disorganization of the medial musculo–elastic structural units and mucoid material deposition. Genetic testing found a variant of unknown significance the PRKG1 gene encoding the protein kinase cGMP-dependent 1, which is important in blood pressure regulation. There may be genetic links between high blood pressure and thoracic aortic aneurysm determinants. Hypertension was found in FBN1 gene mutations encoding fibrillin and in PRKG1 mutations. Possible mechanisms involving the renin–angiotensin system, the role of oxidative stress, osteopontin, epigenetic modifications and other genes are reviewed. Close follow-up and strict hypertension control are required to reduce the risk of dissection. Hypertension, scoliosis and other extra-aortic signs suggesting a connective tissue disease are possible clues for diagnosis.

Thoracic aortic aneurysms in patients with heritable connective tissue disease

2021 ◽  
Vol 36 (3) ◽  
pp. 1083-1090
Author(s):  
Xuan Odofin ◽  
Nour Houbby ◽  
Arwa Hagana ◽  
Ibrahim Nasser ◽  
Amna Ahmed ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Aortic Aneurysms ◽  
Thoracic Aortic Aneurysms

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

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.

Thoracic Aortic Aneurysm Repair with Endovascular Stent-Grafts

1997 ◽  
Vol 2 (2) ◽  
pp. 98-103 ◽  
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.

Elevated expression of connective tissue growth factor, osteopontin and increased collagen content in human ascending thoracic aortic aneurysms

Vascular ◽  
2013 ◽  
Vol 22 (1) ◽  
pp. 20-27 ◽  
Author(s):  
YH Meng ◽  
C Tian ◽  
L Liu ◽  
L Wang ◽  
Q Chang
Keyword(s):  
Growth Factor ◽  
Connective Tissue ◽  
Connective Tissue Growth Factor ◽  
Tissue Growth ◽  
Aortic Aneurysms ◽  
Collagen Content ◽  
Control Group ◽  
Mrna Levels ◽  
Thoracic Aortic Aneurysms ◽  
Protein Levels

Little is known about the molecular mechanisms of ascending thoracic aortic aneurysms (ATAAs). Abnormal extracellular matrix changes and variations of vascular smooth muscle cells (VSMCs) have been implicated in abdominal aortic aneurysm formation. Our objective was to investigate the alterations of collagen, stimulators of collagen synthesis and synthetic VSMCs in patients with ATAA. Surgical samples from ATAA were taken from 20 patients, and 18 control aortas were obtained during coronary artery bypass surgery. All aortic wall specimens were fixed for histology and immunohistochemistry for collagen, connective tissue growth factor (CTGF) and osteopontin. Realtime polymerase chain reaction was used to determine their mRNA expression. Histology and semi-quantitative analysis demonstrated that protein levels of collagen, CTGF and osteopontin significantly increased by 1.9-, 1.4- and 2.2-fold, respectively ( P < 0.01 for all) in the ATAA group than in the control group. Similar results were shown in mRNA levels of type Iα1and IIIα1 collagen, CTGF and osteopontin. The protein levels of CTGF and osteopontin were positively correlated with aortic diameter ( r = 0.67, r = 0.73; P < 0.01 for both). In conclusion, overexpression of aortic CTGF and synthetic VSMCs marker (osteopontin), which is likely to be responsible for elevated aortic collagen content, may provide a potential mechanism for aneurysmal enlargement.

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

2015 ◽  
Vol 4 (1) ◽  
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

Aortic aneurysm: aortic arch aneurysm

ESC CardioMed ◽  
2018 ◽  
pp. 2575-2577
Author(s):  
Roberto Bartolomeo ◽  
Alessandro Leone ◽  
Luca Di Marco ◽  
Davide Pacini
Keyword(s):  
Aortic Aneurysm ◽  
Aortic Arch ◽  
Distal Segment ◽  
Aortic Aneurysms ◽  
Elephant Trunk ◽  
Mortality And Morbidity ◽  
Medial Degeneration ◽  
Hybrid Prosthesis ◽  
Mycotic Aneurysms ◽  
Elephant Trunk Procedure

Thoracic aortic aneurysm (TAA) is defined as aneurysmal degeneration that occurs in the thoracic aorta. The incidence of TAA is increasing with improvements in screening, as well as advances in imaging. They are often asymptomatic but in some cases, they may compress the innominate vein or airway or they may stretch the left recurrent laryngeal nerve, causing hoarseness. TAA often results from cystic medial degeneration and when it occurs at younger ages, it is classically associated with connective tissue disorders, such as Marfan syndrome or, less commonly, Ehlers–Danlos syndrome and Loeys–Dietz syndrome. Mycotic aneurysms, once the predominant cause of ascending and arch aneurysms, are rare today. Diagnosis is often casual and can be suspected on the basis of chest X-ray or as for ascending aortic aneurysms, diagnosed by transthoracic echocardiogram. However, the computed tomography angiography scan represents the gold standard examination for diagnosis. The aortic arch operation consists of the replacement of the arch with reimplantation of the supra-aortic vessels. Effective methods of cerebral, myocardial, as well as visceral protection are necessary to obtain acceptable results in terms of hospital mortality and morbidity. The ‘elephant trunk’ procedure can be an alternative technique for total arch repair; however, a recent evolution of the ‘elephant trunk’ procedure is the ‘frozen elephant trunk’ technique. This technique consists of the implantation of the stented distal segment of the hybrid prosthesis into the descending aorta through the opened aortic arch, while the proximal, non-stented segment is used for conventional replacement of the aortic arch.

scholarly journals Mechanical ventilation variability due to tracheal compression in a patient with a mycotic descending aortic aneurysm: an inverse correlation with blood pressure

2020 ◽  
Vol 58 (4) ◽  
pp. 864-866
Author(s):  
Samuel Heuts ◽  
Roy T M Sprooten ◽  
Serge J H Heines ◽  
Barend M E Mees
Keyword(s):  
Blood Pressure ◽  
Aortic Aneurysm ◽  
Inverse Correlation ◽  
Aortic Aneurysms ◽  
Pressure Management ◽  
Tracheal Compression ◽  
Airway Occlusion ◽  
Risk Of Rupture ◽  
Descending Aortic Aneurysm ◽  
Current Report

Abstract Mycotic aortic aneurysms carry significant morbidity and mortality. In the current report, we present a case of a patient with a mycotic descending aortic aneurysm with contained rupture causing variable compression of the trachea, influenced by a variability in blood pressure. In these patients, blood pressure management is paramount as relative hypertensive periods do not only increase the risk of rupture but can also warrant high ventilation pressures or can potentially result in airway occlusion.

Effects of angiotensin II on remodelling of the airway and the vasculature in the rat

1999 ◽  
Vol 98 (1) ◽  
pp. 1-7 ◽  
Author(s):  
Scott G. RAMSAY ◽  
Christopher J. KENYON ◽  
Niall WHYTE ◽  
Ian C. MCKAY ◽  
Neil C. THOMSON ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Angiotensin Ii ◽  
Young Male ◽  
Renin Angiotensin System ◽  
Mesenteric Arteries ◽  
Airway Remodelling ◽  
Cell Nuclei ◽  
Renin Mrna ◽  
Male Wistar Rats ◽  
Post Infusion

Airway remodelling occurs in chronic asthma. Angiotensin II promotes growth in cardiovascular remodelling. Since the renin–angiotensin system is activated in acute severe asthma, we hypothesized that angiotensin II has a role in airway remodelling. A total of 14 young male Wistar rats were randomly divided into two groups. All received 2-week infusions of bromodeoxyuridine, and the experimental group also received angiotensin II. Blood pressure rose in the angiotensin II-infused group [mean levels: pre-infusion, 134.9 (S.D. 14.7) mmHg; post-infusion, 197.1 (22.5) mmHg], and expression of renin mRNA in the renal juxtaglomerular cells was suppressed in these animals. The proportion of bromodeoxyuridine-positive cell nuclei was no different in the airways of control and angiotensin II-infused animals for smooth muscle [mean bromodeoxyuridine index: control, 8.6% (S.E.M. 1.1%); angiotensin II, 9.3% (1.1%)], epithelium [control, 16.7% (2.3%); angiotensin II, 16.0% (2.2%)] and adventitia [control, 26.4% (2.2%); angiotensin II, 26.6% (2.4%)]. In the arteries, bromodeoxyuridine indices were higher in the angiotensin II-infused rats [18.4% (2.3%)] than in the control animals [9.4% (2.8%)], but no difference was found in the veins [12% (2.9%) and 11.4% (2.6%) respectively]. Morphometry of the airway wall and mesenteric vasculature was no different in the two groups. Therefore a 2-week infusion of angiotensin II increases blood pressure and DNA synthesis in the mesenteric arteries, but does not cause airway remodelling, in the rat.

scholarly journals Nucleoporin p62 Antibodies in a Case of Mixed Connective Tissue Disease

2003 ◽  
Vol 10 (2) ◽  
pp. 329-331 ◽  
Author(s):  
Doris M. Kraemer ◽  
Michael R. Kraus ◽  
Christian Kneitz ◽  
Hans-Peter Tony
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
High Titer ◽  
Young Male ◽  
Severe Case ◽  
Biliary Cirrhosis ◽  
Connective Tissue Disorders ◽  
Systemic Autoimmune Diseases ◽  
U1 Snrnp

ABSTRACT Mixed connective tissue disease is an overlap syndrome characterized by features of different systemic autoimmune diseases and a high titer of U1-snRNP antibodies. We examine here the autoantibodies to nucleoporin p62 in a severe case of mixed connective tissue disease in a young male patient. Thus far, p62 antibodies have mainly been described in cases of primary biliary cirrhosis. We speculate that the presence of p62 antibodies is an indication of a poor prognosis in connective tissue disorders.

scholarly journals The chest pain in neurological practice. Non deesset...

2019 ◽  
Vol 10 (2) ◽  
pp. 91-96
Author(s):  
Elena V. Shirshova ◽  
O. Y. Annenkova ◽  
E. V. Ekusheva ◽  
V. N. Petrov
Keyword(s):  
Chest Pain ◽  
Aortic Aneurysm ◽  
Clinical Manifestations ◽  
Routine Clinical Practice ◽  
Aortic Aneurysms ◽  
Aorta Aneurysm ◽  
Thoracic Aortic Aneurysms ◽  
Threatening Condition ◽  
Life Threatening ◽  
Thoracic Aorta Aneurysm

Chest pain can be a “mask” of a life-threatening condition, which the practitioner must remember. One of such life-threatening condition is aortic aneurysm, which diagnosis presents significant difficulties in routine clinical practice. Clinical manifestations of thoracic aortic aneurysms are extremely variable and non-specific and are mainly depends on the size of the aneurysmal sac, its localization and extent, as well as the etiology of the disease. Here we present a clinical case report of a 48 y.o. patient who died because of the acute cardiac tamponade as a complication of dissecting thoracic aorta aneurysm. The lack of symptoms and clinical instrumental data, initially suggesting the presence of life-threatening disease did not allow the physician to suspect aortic aneurysm and urgently take action regarding it.

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