scholarly journals Mapping Semaphorins and Netrins in the Pathogenesis of Human Thoracic Aortic Aneurysms

2019 ◽  
Vol 20 (9) ◽  
pp. 2100
Author(s):  
Alebrahim ◽  
Nayak ◽  
Ward ◽  
Ursomanno ◽  
Shams ◽  
...  
Keyword(s):  
Internal Thoracic Artery ◽  
Aortic Aneurysms ◽  
Axonal Guidance ◽  
Full Spectrum ◽  
Thoracic Aortic Aneurysms ◽  
Persistent Inflammation ◽  
Novel Association ◽  
Life Threatening ◽  
Ecm Degradation ◽  
Matrix Degrading Enzymes

Thoracic aortic aneurysm (TAA) is a complex life-threatening disease characterized by extensive extracellular matrix (ECM) fragmentation and persistent inflammation, culminating in a weakened aorta. Although evidence suggests defective canonical signaling pathways in TAA, the full spectrum of mechanisms contributing to TAA is poorly understood, therefore limiting the scope of drug-based treatment. Here, we used a sensitive RNA sequencing approach to profile the transcriptomic atlas of human TAA. Pathway analysis revealed upregulation of key matrix-degrading enzymes and inflammation coincident with the axonal guidance pathway. We uncovered their novel association with TAA and focused on the expression of Semaphorins and Netrins. Comprehensive analysis of this pathway showed that several members were differentially expressed in TAA compared to controls. Immunohistochemistry revealed that Semaphorin4D and its receptor PlexinB1, similar to Netrin-1 proteins were highly expressed in damaged areas of TAA tissues but faintly detected in the vessel wall of non-diseased sections. It should be considered that the current study is limited by its sample size and the use of internal thoracic artery as control for TAA for the sequencing dataset. Our data determines important neuronal regulators of vascular inflammatory events and suggest Netrins and Semaphorins as potential key contributors of ECM degradation in TAA.

Ultrasound diagnosis of dissecting thoracic aortic aneurysms: procedure with a handheld device and a video-illustrated case

2021 ◽  
Vol 51 (1) ◽  
pp. 10-15
Author(s):  
Kenneth V Iserson ◽  
Sri Devi Jagjit ◽  
Balram Doodnauth
Keyword(s):  
Aortic Dissection ◽  
Correct Diagnosis ◽  
Signs And Symptoms ◽  
Aortic Aneurysms ◽  
Thoracic Aortic Aneurysms ◽  
Handheld Device ◽  
Thoracic Aortic Dissection ◽  
Threatening Condition ◽  
Life Threatening ◽  
Handheld Ultrasound

Acute thoracic aortic dissection is an uncommon, although not rare, life-threatening condition. With protean signs and symptoms that often suggest more common cardiac or pulmonary conditions, it can be difficult to diagnose. Ultrasound has proven useful in making the correct diagnosis. This case demonstrates that training gained using standard ultrasound machines can be easily and successfully adapted to newer handheld ultrasound devices. The examination technique using the handheld device is illustrated with photos and a video.

scholarly journals Surgical treatment for secondary aortoesophageal fistula

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Kayo Sugiyama ◽  
Toru Iwahashi ◽  
Nobusato Koizumi ◽  
Toshiya Nishibe ◽  
Toshiki Fujiyoshi ◽  
...  
Keyword(s):  
Open Repair ◽  
Rare Condition ◽  
Aortic Aneurysms ◽  
Total Arch Replacement ◽  
University Hospital ◽  
Aortoesophageal Fistula ◽  
Thoracic Aortic Aneurysms ◽  
Life Threatening ◽  
Tokyo Medical ◽  
Medical University

Abstract Background Aortoesophageal fistula (AEF) is a relatively rare condition that is often life-threatening. Secondary AEF is a complication of previous surgery, which can be more critical and challenging than primary AEF. The number of secondary AEF is increasing due to increase in the number of thoracic endovascular aortic repair (TEVAR). Although TEVAR has become a successful alternative surgical strategy for thoracic aortic aneurysms, secondary AEF after TEVAR might be critical than other secondary AEF because of severe adhesion between the esophagus and residual thoracic aortic wall. Methods This study analyzed six patients with secondary AEF who were treated at Tokyo Medical University Hospital between 2011 and 2016. These participants included four patients who had undergone TEVAR and two who had undergone total arch replacement. Results Although they were subsequently hospitalized for a long period, open surgical repair was completed in two patients who had undergone total arch replacement. TEVAR alone was performed in two patients who had undergone TEVAR and they were discharged without major complications shortly. Combined repair of TEVAR as a bridge to open surgery was planned for two patients who had undergone TEVAR. However, reconstruction of the aorta and esophagus could not be completed in these patients due to severe adhesions, and they died during hospitalization. Conclusions Definitive open repair was successfully performed in patients with secondary AEF after total arch replacement. However, in the patients with secondary AEF after TEVAR, severe adhesion between the aorta and esophagus led to difficulty in performing a successful definitive open repair. The strategy for secondary AEF should, therefore, be decided considering the etiology of secondary AEF. In secondary AEF after TEVAR, definitive open repair is difficult to complete because of catastrophic complication, and palliative treatment using TEVAR without reconstruction of aorta and esophagus can be an alternative.

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.

scholarly journals Novel LTBP3 mutations associated with thoracic aortic aneurysms and dissections

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Guoyan Zhu ◽  
Mingyao Luo ◽  
Qianlong Chen ◽  
Yinhui Zhang ◽  
Kun Zhao ◽  
...  
Keyword(s):  
Case Reports ◽  
Asian Population ◽  
Aortic Aneurysms ◽  
Compound Heterozygous ◽  
Thoracic Aortic Aneurysms ◽  
Functional Studies ◽  
Pathogenic Variants ◽  
Aortic Aneurysm And Dissection ◽  
Life Threatening ◽  
Full Knowledge

Abstract Background Thoracic aortic aneurysm and dissection (TAAD) is a hidden-onset but life-threatening disorder with high clinical variability and genetic heterogeneity. In recent years, an increasing number of genes have been identified to be related to TAAD. However, some genes remain uncertain because of limited case reports and/or functional studies. LTBP3 was such an ambiguous gene that was previously known for dental and skeletal dysplasia and then noted to be associated with TAAD. More research on individuals or families harboring variants in this gene would be helpful to obtain full knowledge of the disease and clarify its association with TAAD. Methods A total of 266 TAAD probands with no causative mutations in known genes had been performed wholeexome sequencing (WES) to identify potentially pathogenic variants. In this study, rare LTBP3 variants were the focus of analysis. Results Two compound heterozygous mutations, c.625dup (p.Leu209fs) and c.1965del (p.Arg656fs), in LTBP3 were identified in a TAAD patient along with short stature and dental problems, which was the first TAAD case with biallelic LTBP3 null mutations in an Asian population. Additionally, several rare heterozygous LTBP3 variants were also detected in other sporadic TAAD patients. Conclusion The identification of LTBP3 mutations in TAAD patients in our study provided more clinical evidence to support its association with TAAD, which broadens the gene spectrum of LTBP3. LTBP3 should be considered to be incorporated into the routine genetic analysis of heritable aortopathy, which might help to fully understand its phenotypic spectrum and improve the diagnostic rate of TAAD.

Association of Fiber Orientation and Dissection Properties of Ascending Thoracic Aortic Aneurysms With Aortic Valve Morphology

2011 ◽  
Author(s):  
Salvatore Pasta ◽  
Julie A. Phillippi ◽  
Antonio D’Amore ◽  
Michael S. Sacks ◽  
Simon C. Watkins ◽  
...  
Keyword(s):  
Aortic Dissection ◽  
Acute Aortic Dissection ◽  
Surgical Techniques ◽  
Aortic Aneurysms ◽  
Type A Aortic Dissection ◽  
Thoracic Aortic Aneurysms ◽  
International Registry ◽  
Life Threatening ◽  
Morbidity Risk ◽  
Emergent Surgery

Type A aortic dissection (AoD) of an ascending thoracic aortic aneurysm (ATAA) is a life-threatening cardiovascular emergency with a high potential for death. Despite improved surgical techniques, the morbidity risk for emergent surgery remains 24% worldwide according to data from the International Registry of Acute Aortic Dissection [1].

scholarly journals Insights on the Pathogenesis of Aneurysm through the Study of Hereditary Aortopathies

Genes ◽  
2021 ◽  
Vol 12 (2) ◽  
pp. 183
Author(s):  
Tyler J. Creamer ◽  
Emily E. Bramel ◽  
Elena Gallo MacFarlane
Keyword(s):  
Transforming Growth Factor ◽  
Transforming Growth Factor Β ◽  
Aortic Aneurysms ◽  
Gene Products ◽  
Molecular Processes ◽  
Thoracic Aortic Aneurysms ◽  
Pathogenic Variants ◽  
The Matrix ◽  
Life Threatening ◽  
Causal Variants

Thoracic aortic aneurysms (TAA) are permanent and localized dilations of the aorta that predispose patients to a life-threatening risk of aortic dissection or rupture. The identification of pathogenic variants that cause hereditary forms of TAA has delineated fundamental molecular processes required to maintain aortic homeostasis. Vascular smooth muscle cells (VSMCs) elaborate and remodel the extracellular matrix (ECM) in response to mechanical and biochemical cues from their environment. Causal variants for hereditary forms of aneurysm compromise the function of gene products involved in the transmission or interpretation of these signals, initiating processes that eventually lead to degeneration and mechanical failure of the vessel. These include mutations that interfere with transduction of stimuli from the matrix to the actin–myosin cytoskeleton through integrins, and those that impair signaling pathways activated by transforming growth factor-β (TGF-β). In this review, we summarize the features of the healthy aortic wall, the major pathways involved in the modulation of VSMC phenotypes, and the basic molecular functions impaired by TAA-associated mutations. We also discuss how the heterogeneity and balance of adaptive and maladaptive responses to the initial genetic insult might contribute to disease.

scholarly journals Surgical treatment for secondary aortoesophageal fistula

2020 ◽  
Author(s):  
Kayo Sugiyama ◽  
Toru Iwahashi ◽  
Nobusato Koizumi ◽  
Toshiya Nishibe ◽  
Toshiki Fujiyoshi ◽  
...  
Keyword(s):  
Open Repair ◽  
Rare Condition ◽  
Aortic Aneurysms ◽  
Total Arch Replacement ◽  
University Hospital ◽  
Aortoesophageal Fistula ◽  
Thoracic Aortic Aneurysms ◽  
Life Threatening ◽  
Tokyo Medical ◽  
Medical University

Abstract BackgroundAortoesophageal fistula (AEF) is a relatively rare condition that is often life-threatening. Secondary AEF is a complication of previous surgery, which can be more critical and challenging than primary AEF. The number of secondary AEF is increasing due to increase in the number of thoracic endovascular aortic repair (TEVAR). Although TEVAR has become a successful alternative surgical strategy for thoracic aortic aneurysms, secondary AEF after TEVAR might be critical than other secondary AEF because of severe adhesion between the esophagus and residual thoracic aortic wall.MethodsThis study analyzed six patients with secondary AEF who were treated at Tokyo Medical University Hospital between 2011 and 2016. These participants included four patients who had undergone TEVAR and two who had undergone total arch replacement.ResultsAlthough they were subsequently hospitalized for a long period, open surgical repair was completed in two patients who had undergone total arch replacement. TEVAR alone was performed in two patients who had undergone TEVAR and they were discharged without major complications shortly. Combined repair of TEVAR as a bridge to open surgery was planned for two patients who had undergone TEVAR. However, reconstruction of the aorta and esophagus could not be completed in these patients due to severe adhesions, and they died during hospitalization.ConclusionsDefinitive open repair was successfully performed in patients with secondary AEF after total arch replacement. However, in the patients with secondary AEF after TEVAR, severe adhesion between the aorta and esophagus led to difficulty in performing a successful definitive open repair. The strategy for secondary AEF should, therefore, be decided considering the etiology of secondary AEF. In secondary AEF after TEVAR, definitive open repair is difficult to complete because of catastrophic complication, and palliative treatment using TEVAR without reconstruction of aorta and esophagus can be an alternative.

scholarly journals Thoracic aortic aneurysms exerting high extrinsic pressure on the airway

2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Hanna Jung ◽  
Young Woo Do ◽  
Sang Yub Lee ◽  
Youngok Lee ◽  
Tak Hyuk Oh ◽  
...  
Keyword(s):  
Thoracic Aortic Aneurysm ◽  
Thoracic Endovascular Aortic Repair ◽  
Aortic Aneurysms ◽  
Case Presentation ◽  
Descending Thoracic Aortic Aneurysm ◽  
Thoracic Aortic Aneurysms ◽  
Technological Improvement ◽  
Risk Of Rupture ◽  
Lower Trachea ◽  
Life Threatening

Abstract Background Thoracic aortic aneurysms, although mostly asymptomatic, are life threatening owing to the risk of rupture. Moreover, the extrinsic pressure of a ruptured aneurysm may encroach the mediastinum. Case presentation A 74-year-old woman diagnosed with ruptured descending thoracic aortic aneurysm compressing the lower trachea and both main bronchi underwent thoracic endovascular aortic repair; however, the extrinsic pressure on the airway persisted. Following failing of endobronchial silicon stents insertion, extracorporeal membrane oxygenation support was required, and endobronchial metallic stents were inserted. The patients’ hypoventilation resolved, and the patient was withdrawn from the ventilator. Conclusions Technological improvement in endovascular or endobronchial procedures has provided more options for managing complex cases. However, we must be aware of how high the extrinsic pressure might be before management and take steps to minimize complications.

TEVAR—Thoracic Endovascular Surgery—Cerebral Spinal Fluid Drainage Catheters

2018 ◽  
pp. 95-102
Author(s):  
Oran Kreme ◽  
Kumar Vivek
Keyword(s):  
Spinal Cord ◽  
Cerebral Spinal Fluid ◽  
Spinal Cord Ischemia ◽  
Aortic Aneurysms ◽  
Significant Morbidity ◽  
Neurologic Injury ◽  
New Techniques ◽  
Thoracic Aortic Aneurysms ◽  
Life Threatening ◽  
Lumbar Spinal

Thoracic aortic aneurysms (TAA) are potentially life-threatening conditions that must be monitored closely. Aortic aneurysms can arise at all levels of the aorta and are associated with several medical conditions and congenital syndromes. Aneurysms can progress to acute dissections and/or rupture, requiring emergent intervention. Classically, TAAs were repaired with large thoracotomies, but new techniques and technologies allow these aneurysms to be repaired by endovascular technique. Even though these techniques are minimally invasive, they still carry significant morbidity and mortality, including devastating neurologic injury from spinal cord ischemia. Lumbar spinal drains and other techniques are being used to prevent neurologic injury and improve the outcomes of TAA repairs.

Staged Repair of a Ruptured Thoracic Aortic Aneurysm Having an Aorto-Parenchymal Fistula

2017 ◽  
Vol 12 (5) ◽  
pp. e13-e15 ◽  
Author(s):  
Akihiro Yoshitake ◽  
Kazuma Okamoto ◽  
Mio Kasai ◽  
Hideyuki Shimizu
Keyword(s):  
Aortic Aneurysm ◽  
Thoracic Aortic Aneurysm ◽  
Thoracic Endovascular Aortic Repair ◽  
Aortic Aneurysms ◽  
Treatment Results ◽  
Term Survival ◽  
Thoracic Aortic Aneurysms ◽  
Second Stage ◽  
Life Threatening

Ruptured thoracic aortic aneurysms, having aorto-bronchial or pulmonary parenchymal fistulations, are life-threatening conditions, and current surgical treatment results are not satisfactory. Herein, we describe a 69-year-old man with a ruptured thoracic aortic aneurysm and aorto-pulmonary parenchymal fistulation that was successfully treated using a staged repair. The first stage involved an emergency thoracic endovascular aortic repair for salvage and the second stage involved radical pulmonary parenchymal repair with removal of the stent graft and aortic reconstruction for long-term survival.

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