scholarly journals Giant Aortic Aneurysm in Child with Cutis Laxa Syndrome: Unusual Presentation, New Surgical Technique

2021 ◽  
Vol 24 (6) ◽  
pp. E1054-E1056
Author(s):  
Mazen Shamsaldeen Faden ◽  
Nada Ahmed Noaman ◽  
Osman Osama Osman Osama ◽  
Ahmed Abdelrahman Elassal ◽  
Arwa Mohammed Al-ghamdi ◽  
...  
Keyword(s):  
Final Diagnosis ◽  
Ascending Aorta ◽  
Accurate Diagnosis ◽  
Aortic Aneurysms ◽  
Cutis Laxa ◽  
Descending Aorta ◽  
Aorta Aneurysm ◽  
Thoracic Aortic Aneurysms ◽  
Valvular Insufficiency ◽  
New Surgical Technique

Ascending thoracic aortic aneurysms are rare in childhood and typically are seen in the setting of connective tissue defect syndromes. These aneurysms may lead to rupture, dissection, or valvular insufficiency, so root replacement is recommended. Here, we present a 17-month-old girl who presented with fever, cough, and pericardial effusion. Initially, we suspected this could be a COVID-19 case, so a nasopharyngeal swap was performed. An ascending aorta aneurysm involving the aortic arch was confirmed by echo, and urgent ascending aorta and arch replacement were done by utilizing the descending aorta as a new arch. The final diagnosis came with cutis laxa syndrome. In similar cases, good outcomes can be achieved with accurate diagnosis and appropriate surgical management.

Cannulation of the Ascending Aorta in Left Thoracotomy for Thoracic Aortic Aneurysms

2007 ◽  
Vol 10 (1) ◽  
pp. E81-E83 ◽  
Author(s):  
Riza Turkoz ◽  
Oner Gulcan ◽  
Orhan Demirturk ◽  
Ayda Turkoz
Keyword(s):  
Ascending Aorta ◽  
Aortic Aneurysms ◽  
Left Thoracotomy ◽  
Thoracic Aortic Aneurysms

scholarly journals Comparison of 10 murine models reveals a distinct biomechanical phenotype in thoracic aortic aneurysms

2017 ◽  
Vol 14 (130) ◽  
pp. 20161036 ◽  
Author(s):  
C. Bellini ◽  
M. R. Bersi ◽  
A. W. Caulk ◽  
J. Ferruzzi ◽  
D. M. Milewicz ◽  
...  
Keyword(s):  
Extracellular Matrix ◽  
Structural Integrity ◽  
Ascending Aorta ◽  
Elastic Fibre ◽  
Cytoskeletal Proteins ◽  
Aortic Aneurysms ◽  
Genetic Mutations ◽  
Murine Models ◽  
Thoracic Aortic Aneurysms ◽  
Signalling Molecules

Thoracic aortic aneurysms are life-threatening lesions that afflict young and old individuals alike. They frequently associate with genetic mutations and are characterized by reduced elastic fibre integrity, dysfunctional smooth muscle cells, improperly remodelled collagen and pooled mucoid material. There is a pressing need to understand better the compromised structural integrity of the aorta that results from these genetic mutations and renders the wall vulnerable to dilatation, dissection or rupture. In this paper, we compare the biaxial mechanical properties of the ascending aorta from 10 murine models: wild-type controls, acute elastase-treated, and eight models with genetic mutations affecting extracellular matrix proteins, transmembrane receptors, cytoskeletal proteins, or intracellular signalling molecules. Collectively, our data for these diverse mouse models suggest that reduced mechanical functionality, as indicated by a decreased elastic energy storage capability or reduced distensibility, does not predispose to aneurysms. Rather, despite normal or lower than normal circumferential and axial wall stresses, it appears that intramural cells in the ascending aorta of mice prone to aneurysms are unable to maintain or restore the intrinsic circumferential material stiffness, which may render the wall biomechanically vulnerable to continued dilatation and possible rupture. This finding is consistent with an underlying dysfunctional mechanosensing or mechanoregulation of the extracellular matrix, which normally endows the wall with both appropriate compliance and sufficient strength.

scholarly journals Primary Lymphoma Presenting in an Ascending Aortic Aneurysm: A Case Report

Aorta ◽  
2020 ◽  
Vol 08 (02) ◽  
pp. 038-040
Author(s):  
Alexander M. Schurman ◽  
David Mendoza ◽  
Chris K. Rokkas
Keyword(s):  
Management Strategies ◽  
Ascending Aorta ◽  
Aortic Aneurysms ◽  
Prior History ◽  
Primary Lymphoma ◽  
Surgical Specimen ◽  
Hematological Disorders ◽  
Thoracic Aortic Aneurysms ◽  
Asymptomatic Patients ◽  
History Of

AbstractSmall lymphocytic lymphoma (SLL) is rarely associated with thoracic aortic aneurysms. Aneurysm of the ascending aorta associated with SLL has never been reported before. We describe the case of an asymptomatic 68-year-old woman who presented with a 5.5-cm aneurysm of the ascending aorta and no prior history of hematological disorders. Following excision and repair, the surgical specimen showed infiltration of the aortic wall by lymphocytes, expressing markers consistent with SLL. While symptomatic SLL carries a poor prognosis, risk stratification tools are applied to guide management strategies in asymptomatic patients.

Ascending Thoracic Aorta Exhibits Anisotropic Failure Behavior in Shear Lap Testing

2013 ◽  
Author(s):  
Colleen Witzenburg ◽  
Sachin Shah ◽  
Hallie P. Wagner ◽  
Janna Goodrich ◽  
Victor H. Barocas
Keyword(s):  
Arterial Pressure ◽  
Mechanical Behavior ◽  
Thoracic Aorta ◽  
Ascending Aorta ◽  
Aortic Aneurysms ◽  
Failure Behavior ◽  
Thoracic Aortic Aneurysms ◽  
Wall Strength ◽  
Imminent Death ◽  
Aneurysm Dissection

Aneurysm dissection and rupture, resulting in imminent death, is the primary risk associated with thoracic aortic aneurysms (TAA). Nearly 60% of TAA involves the ascending aorta [1]. Dissection and rupture occur when the remodeled tissue is no longer able to withstand the stresses generated by the arterial pressure. As the ascending TAA grows, however, changes in its mechanical behavior, particularly wall strength, are unknown.

scholarly journals Case Report: Occurrence of Severe Thoracic Aortic Aneurysms (Involving the Ascending, Arch, and Descending Segments) as a Result of Fibulin-4 Deficiency: A Rare Pathology With Successful Management

2021 ◽  
Vol 8 ◽  
Author(s):  
Paul Thomas ◽  
Aparna Venugopalan ◽  
Siddharth Narayanan ◽  
Thomas Mathew ◽  
Lakshmi Parvathi Deepti Cherukuwada ◽  
...  
Keyword(s):  
Matrix Protein ◽  
Genetic Disorder ◽  
Female Child ◽  
Aortic Aneurysms ◽  
Cutis Laxa ◽  
Extracellular Matrix Protein ◽  
Successful Outcome ◽  
High Morbidity ◽  
Aortic Diseases ◽  
Thoracic Aortic Aneurysms

Aortic diseases requiring surgery in childhood are distinctive and rare. Very few reports in the literature account for the occurrence of multiple thoracic aortic aneurysms in the same pediatric patient because of a genetic cause. We report a rare occurrence of severe thoracic aortic aneurysms (involving the ascending, arch and descending aortic segments) with severe aortic insufficiency in a 7-year-old female child secondary to the extremely rare and often lethal genetic disorder, cutis laxa. She was eventually identified as a carrier of a homozygous EFEMP2 (alias FBLN4) mutation. This gene encodes the extracellular matrix protein fibulin-4, and its mutation is associated with autosomal recessive cutis laxa type 1B that leads to severe aortopathy with aneurysm formation and vascular tortuosity. Parents of the child were not known to be consanguineous. Significant symptomatic improvement in the patient could be discerned after timely intervention with the valve-sparing aortic root replacement (David V procedure) and a concomitant aortic arch replacement. This is a unique report with a successful outcome that highlights the occurrence of a rare hereditary aortopathy associated with a high morbidity and mortality, and the importance of an early diagnosis and timely management. It also offers insight to physicians in having a very broad differential and multimodal approach in handling rare pediatric cardio-pathologies with a genetic predisposition.

scholarly journals Extra-anatomic thoracic aortic bypass operations

Medicina ◽  
2008 ◽  
Vol 44 (5) ◽  
pp. 373
Author(s):  
Arūnas Valaika ◽  
Gediminas Norkūnas ◽  
Gintaras Kalinauskas ◽  
Giedrė Nogienė ◽  
Jurgis Verižnikovas ◽  
...  
Keyword(s):  
Aortic Dissection ◽  
Abdominal Aorta ◽  
Bypass Graft ◽  
Emergency Operation ◽  
Aortic Aneurysms ◽  
Major Surgery ◽  
Descending Aorta ◽  
Aorta Aneurysm ◽  
Aortic Bypass ◽  
Thoracic Aneurysms

Objective. When the patient condition contraindicates major surgery for descending thoracic aneurysms, the surgeon should consider using an ascending aorta to abdominal aorta bypass graft, leaving the diseased segment undisturbed. Our experience with eight patients is presented. Material and methods. Between 1988 and 2008, eight patients were treated for the following indications: reoperation for coarctation (two patients), complicated descending aortic aneurysms (five patients), and posttraumatic descending aorta dissection (one patient). The mean age of the patients was 44±8 years (range, 27–53 years). There were 6 (75%) males and 2 (25%) females. Emergency operations were performed in three patients (two with aortic recoarctation, one with posttraumatic aortic dissection). Two cases were reoperations (both after recoarctation). Descending aorta was ligated in seven cases. Distal anastomosis was connected with abdominal aorta in four cases and with iliac arteries in four patients. Results. Three early deaths occurred. Two patients died after emergency operation after recoarctation and posttraumatic aortic dissection, and one patient died after descending aorta aneurysm correction because of bleeding. Conclusions. In complex aortic coarctation, extra-anatomic bypass operation remains an effective procedure. The usage of these procedures in patients with descending aortic aneurysms remains complicated.

Ascending aorta mechanics in bicuspid aortopathy: controversy or fact?

2020 ◽  
pp. 021849232092873
Author(s):  
Dimitrios C Iliopoulos ◽  
Dimitrios P Sokolis
Keyword(s):  
Mechanical Properties ◽  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Ascending Aorta ◽  
Aortic Aneurysms ◽  
Aortic Dilatation ◽  
Aged Patients ◽  
Thoracic Aortic Aneurysms ◽  
Maximal Diameter ◽  
Sole Criterion

Bicuspid aortic valve is the most common congenital cardiovascular defect, often associated with proximal aortic dilatation, and the ideal management strategy is debated. The inconsistency in previous and present guideline recommendations emphasizes the insufficiency of the maximal diameter as the sole criterion for prophylactic repair. Our ability to guide clinical decisions may improve through an understanding of the mechanical properties of ascending thoracic aortic aneurysms in bicuspid compared to tricuspid aortic valve patients and non-aneurysmal aortas, because dissection and rupture are aortic wall mechanical failures. Such an understanding of the mechanical properties has been attempted by several authors, and this article addresses whether there is a controversy in the accumulated knowledge. The available mechanical studies are briefly reviewed, discussing factors such as age, sex, and the region of mechanical examination that may be responsible for the lack of unanimity in the reported findings. The rationale for acquiring layer-specific properties is presented along with the main results from our recent study. No mechanical vulnerability of ascending thoracic aortic aneurysms was evidenced in bicuspid aortic valve patients, corroborating present conservative guidelines concerning the management of bicuspid aortopathy. Weakening and additional vulnerability was evidenced in aged patients and those with coexisting valve pathology, aortic root dilatation, hypertension, and hyperlipidemia. Discussion of these results from age- and sex-matched subjects, accounting for the region- and layer-specific aortic heterogeneity, in relation to intact wall results and histologic confirmation, helps to reconcile previous findings and affords a universal interpretation of ascending aorta mechanics in bicuspid aortopathy.

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 Extrinsic tricuspid valve compression due to an aortic aneurysm causing significant right to left shunt via a patent foramen ovale: a case report

2020 ◽  
Author(s):  
Rizwan Ahmed
Keyword(s):  
Aortic Aneurysm ◽  
Tricuspid Valve ◽  
Patent Foramen Ovale ◽  
Ambient Air ◽  
Ascending Aorta ◽  
Aortic Aneurysms ◽  
Foramen Ovale ◽  
Aorta Aneurysm ◽  
Ct Angiogram ◽  
Adjacent Structures

Abstract Background Aortic aneurysms are known to cause compression of adjacent structures including the tracheobronchial tree, oesophagus, and recurrent laryngeal nerve. Extremely rarely, they can lead to compression of the tricuspid valve (TV) annulus. We describe a case where aortic aneurysm caused TV annulus compression and persistent right-to-left shunt through a patent foramen ovale (PFO). Case summary A 75-year-old female was admitted with headache and dizziness. On examination, she had persistent arterial desaturation with oxygen levels reduced to 69% at rest whilst breathing ambient air. Complete blood count demonstrated polycythaemia (Hb 174 g/L). Right to left cardiac shunt was suspected after significant lung and haematologic pathology was excluded. Transoesophageal echocardiography demonstrated a trileaflet aortic valve with an ascending aorta aneurysm and a stretched PFO with persistent right to left shunt across it. The ascending aortic aneurysm was observed coursing superior to and compressing the TV annulus. Invasive haemodynamic data demonstrated prominent ‘a’ waves in the right atrium, low RV (12/1 mmHg), and pulmonary artery pressures (14/6 mmHg), reduced cardiac output and significant right to left shunt with Qp:Qs 0.6. Computed tomography (CT) angiogram demonstrated a 5 cm fusiform ascending aorta aneurysm that coursed anteriorly causing TV annulus compression. Discussion Tricuspid valve inflow obstruction associated with a right to left shunt across PFO can be an extremely rare complication of aortic aneurysm. This may result in persistent arterial hypoxaemia and secondary polycythaemia.

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