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Balkrishna A, Mishra S, Rana M, Rajput SK, Pathak S, Liu K, Dhanasekaran M, Arya V, Singh S. Small molecule inhibitors target multiple neuropathological signaling to exert novel neuroprotection in intracranial aneurysms. Front Pharmacol 2024; 15:1469211. [PMID: 39575394 PMCID: PMC11578703 DOI: 10.3389/fphar.2024.1469211] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/23/2024] [Accepted: 09/30/2024] [Indexed: 11/24/2024] Open
Abstract
Intracranial aneurysms (IAs) represent a critical health concern due to their potential to rupture, leading to severe morbidity and mortality. Small molecule inhibitors (SMIs) have emerged as promising therapeutic candidates for managing IA progression and rupture risk. The current landscape of SMIs targets various molecular pathways implicated in IA pathogenesis, including inflammation, endothelial dysfunction, and extracellular matrix (ECM) degradation. Among the prominent therapeutic candidates discussed are statins, recognized for their multifaceted effects, anti-inflammatory properties, and enhancement of endothelial stability, which may mitigate IA progression. Matrix metalloproteinase inhibitors are also highlighted for their role in preserving ECM structural integrity, essential for preventing IA wall weakening and rupture. Furthermore, the review evaluates the efficacy of anti-inflammatory agents such as corticosteroids and cytokine inhibitors in attenuating IA growth driven by inflammatory processes. Our findings highlight the possibility of several pharmaceutical therapies that target matrix remodeling, inflammation, and other underlying processes to manage cerebral aneurysms. By precisely delivering therapeutic chemicals, such as antioxidants, gene therapy vectors, or anti-inflammatory medicines, to the aneurysm site, these SMI technologies treat the underlying pathophysiological causes while sparing healthy brain tissue. This review underscores the potential of SMIs as adjunctive or primary therapies in the comprehensive management of IAs, emphasizing the need for further clinical research to optimize their efficacy and safety in clinical practice.
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Affiliation(s)
- Acharya Balkrishna
- Patanjali Herbal Research Department, Patanjali Research Foundation, Haridwar, India
| | - Shalini Mishra
- Patanjali Herbal Research Department, Patanjali Research Foundation, Haridwar, India
| | - Maneesha Rana
- Patanjali Herbal Research Department, Patanjali Research Foundation, Haridwar, India
| | - Satyendra Kumar Rajput
- Department of Pharmaceutical Sciences, Gurukula Kangri (Deemed to be University), Haridwar, India
| | - Suhrud Pathak
- Department of Drug Discovery and Development, Harrison College of Pharmacy, Auburn University, Auburn, AL, United States
| | - Keyi Liu
- Department of Drug Discovery and Development, Harrison College of Pharmacy, Auburn University, Auburn, AL, United States
| | - Muralikrishnan Dhanasekaran
- Department of Drug Discovery and Development, Harrison College of Pharmacy, Auburn University, Auburn, AL, United States
| | - Vedpriya Arya
- Patanjali Herbal Research Department, Patanjali Research Foundation, Haridwar, India
| | - Shalini Singh
- Department of Pharmaceutical Sciences, Gurukula Kangri (Deemed to be University), Haridwar, India
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2
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Pucci A, Rossetti M, Lenzi C, Buja ML. The cardiovascular pathologist in the aortic team. Cardiovasc Pathol 2024; 72:107649. [PMID: 38703970 DOI: 10.1016/j.carpath.2024.107649] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/11/2023] [Revised: 04/14/2024] [Accepted: 04/19/2024] [Indexed: 05/06/2024] Open
Abstract
Aortic diseases require a multidisciplinary management for diagnosis, treatment and follow-up with better outcomes in referral centers using a team-based approach. The setting up of a multi-disciplinary aortic team for the discussion of complex cases has been already proposed; it is also supported by the ACC/AHA. Surgeons and radiologists, more or less other physicians such as cardiologists, geneticists, rheumatologists/internal medicine specialists and pathologists are involved into such a team. The role of the cardiovascular pathologist is to examine the aortic specimens, to diagnose and classify the aortic lesions. Herein, the role of the pathologist in the aortic team is discussed and the pathobiology of aortic diseases is reviewed for reference by pathologists. The aortic specimens are mainly obtained from emergency or elective surgical procedures on the thoracic aorta, less frequently from organ/tissue (including cardiac or heart valve) donors, post-mortem procedures or abdominal aortic surgery. In the last decade, together with the progress of medical sciences, the histological definitions and classifications of the aortic pathology are undergoing thorough revisions that are addressed to an etiopathogenetic approach because of possible clinico-pathological correlations, therapeutic and prognostic impact. Pathologists may also have an important role in research and teaching. Therefore, histological analyses of the aortic specimens require adequate sample processing and pathologist expertise because histology contributes to definite diagnosis, correct management of patients and even (in genetic diseases) families, but also to research in the challenging field of aortopathies.
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Affiliation(s)
- Angela Pucci
- Department of Histopathology, Pisa University Hospital, Pisa, Italy.
| | - Martina Rossetti
- Department of Histopathology, Pisa University Hospital, Pisa, Italy
| | - Chiara Lenzi
- Department of Histopathology, Pisa University Hospital, Pisa, Italy
| | - Maximilian L Buja
- Department of Pathology and Laboratory Medicine, McGovern Medical School, The University of Texas Health Science Center at Houston (UTHealth), Houston, TX, USA
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3
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Barkhordarian M, Tran HHV, Menon A, Pulipaka SP, Aguilar IK, Fuertes A, Dey S, Chacko AA, Sethi T, Bangolo A, Weissman S. Innovation in pathogenesis and management of aortic aneurysm. World J Exp Med 2024; 14:91408. [PMID: 38948412 PMCID: PMC11212750 DOI: 10.5493/wjem.v14.i2.91408] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/28/2023] [Revised: 02/04/2024] [Accepted: 03/18/2024] [Indexed: 06/19/2024] Open
Abstract
Aortic aneurysm (AA) refers to the persistent dilatation of the aorta, exceeding three centimeters. Investigating the pathophysiology of this condition is important for its prevention and management, given its responsibility for more than 25000 deaths in the United States. AAs are classified based on their location or morphology. various pathophysiologic pathways including inflammation, the immune system and atherosclerosis have been implicated in its development. Inflammatory markers such as transforming growth factor β, interleukin-1β, tumor necrosis factor-α, matrix metalloproteinase-2 and many more may contribute to this phenomenon. Several genetic disorders such as Marfan syndrome, Ehler-Danlos syndrome and Loeys-Dietz syndrome have also been associated with this disease. Recent years has seen the investigation of novel management of AA, exploring the implication of different immune suppressors, the role of radiation in shrinkage and prevention, as well as minimally invasive and newly hypothesized surgical methods. In this narrative review, we aim to present the new contributing factors involved in pathophysiology of AA. We also highlighted the novel management methods that have demonstrated promising benefits in clinical outcomes of the AA.
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Affiliation(s)
- Maryam Barkhordarian
- Department of Internal Medicine, Palisades Medical Center, North Bergen, NJ 07047, United States
| | - Hadrian Hoang-Vu Tran
- Department of Internal Medicine, Palisades Medical Center, North Bergen, NJ 07047, United States
| | - Aiswarya Menon
- Department of Internal Medicine, Palisades Medical Center, North Bergen, NJ 07047, United States
| | - Sai Priyanka Pulipaka
- Department of Internal Medicine, Palisades Medical Center, North Bergen, NJ 07047, United States
| | - Izage Kianifar Aguilar
- Department of Internal Medicine, Palisades Medical Center, North Bergen, NJ 07047, United States
| | - Axel Fuertes
- Department of Internal Medicine, Palisades Medical Center, North Bergen, NJ 07047, United States
| | - Shraboni Dey
- Department of Internal Medicine, Palisades Medical Center, North Bergen, NJ 07047, United States
| | - Angel Ann Chacko
- Department of Internal Medicine, Palisades Medical Center, North Bergen, NJ 07047, United States
| | - Tanni Sethi
- Department of Internal Medicine, Palisades Medical Center, North Bergen, NJ 07047, United States
| | - Ayrton Bangolo
- Department of Internal Medicine, Palisades Medical Center, North Bergen, NJ 07047, United States
| | - Simcha Weissman
- Department of Internal Medicine, Palisades Medical Center, North Bergen, NJ 07047, United States
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4
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Buksh MM, Nuzhath S, Heslop J, Moawad M. A systematic review and case presentation: Giant abdominal aortic aneurysm. Vascular 2024; 32:521-532. [PMID: 36598291 DOI: 10.1177/17085381221140166] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/05/2023]
Abstract
OBJECTIVE An abdominal aortic aneurysm is considered giant when its transverse diameter is greater than 10-13 cm in diameter. A giant abdominal aortic aneurysm is rare but with a significant risk of rupture if it is not diagnosed or left untreated. METHOD The authors have performed a systematic review of the evidence that has looked into the clinical presentations, and management methods employed and have presented a 14 cm giant abdominal aortic aneurysm patient. RESULTS The systematic review has been based on level-IV evidence due to the rarity of the condition. The final analysis included 61 relevant reported cases. The mean age was 72.4 years, the male to female ratio was 52: 8, and the average size of a giant abdominal aortic aneurysm was 14.7 cm. These were mostly infra renal (72.58%). Rupture of these aneurysms was found in 23 (37.1%) patients, and was treated by laparotomy in 51 (82.25%) cases. There were 11 (17.74%) mortalities. CONCLUSION The size of an abdominal aortic aneurysm is known to be the biggest factor in the rupture of an aneurysm. The reason abdominal aortic aneurysms can reach such size without rupturing is unclear but needs further exploring. Early diagnosis with effective screening programmes is essential to diagnose in a timely manner to avoid life-threatening consequences.
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Affiliation(s)
- Md Mahfooz Buksh
- General Surgery, Ashford and St Peters Hospital NHS, Chertsey, UK
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5
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Jabagi H, Levine D, Gharibeh L, Camillo C, Castillero E, Ferrari G, Takayama H, Grau JB. Implications of Bicuspid Aortic Valve Disease and Aortic Stenosis/Insufficiency as Risk Factors for Thoracic Aortic Aneurysm. Rev Cardiovasc Med 2023; 24:178. [PMID: 39077527 PMCID: PMC11264121 DOI: 10.31083/j.rcm2406178] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/07/2023] [Revised: 03/27/2023] [Accepted: 04/03/2023] [Indexed: 07/31/2024] Open
Abstract
Bicuspid Aortic Valves (BAV) are associated with an increased incidence of thoracic aortic aneurysms (TAA). TAA are a common aortic pathology characterized by enlargement of the aortic root and/or ascending aorta, and may become life threatening when left untreated. Typically occurring as the sole pathology in a patient, TAA are largely asymptomatic. However, in some instances, they are accompanied by aortic valve (AV) diseases: either congenital BAV or acquired in the form of Aortic Insufficiency (AI) or aortic stenosis (AS). When TAA are associated with aortic valve disease, determining an accurate and predictable prognosis becomes especially challenging. Patients with AV disease and concomitant TAA lack a widely accepted diagnostic approach, one that integrates our knowledge on aortic valve pathophysiology and encompasses multi-modality imaging approaches. This review summarizes the most recent scientific knowledge regarding the association between AV diseases (BAV, AI, AS) and ascending aortopathies (dilatation, aneurysm, and dissection). We aimed to pinpoint the gaps in monitoring practices and prediction of disease progression in TAA patients with concomitant AV disease. We propose that a morphological and functional analysis of the AV with multi-modality imaging should be included in aortic surveillance programs. This strategy would allow for improved risk stratification of these patients, and possibly new AV phenotypic-specific guidelines with more vigilant surveillance and earlier prophylactic surgery to improve patient outcomes.
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Affiliation(s)
- Habib Jabagi
- Division of Cardiothoracic Surgery, The Valley Hospital, NJ 07450, USA
- Department of Cardiovascular Surgery, Mt. Sinai Hospital, Icahn School of Medicine, New York, NY 10029, USA
| | - Dov Levine
- Department of Surgery, Columbia University, New York, NY 10027, USA
| | - Lara Gharibeh
- Department of Biochemistry, Microbiology and Immunology, University of Ottawa, Ottawa, ON K1N 6N5, Canada
- Division of Cardiac Surgery, University of Ottawa Heart Institute, Ottawa, ON K1Y 4W7, Canada
| | - Chiara Camillo
- Department of Surgery, Columbia University, New York, NY 10027, USA
| | | | - Giovanni Ferrari
- Department of Surgery, Columbia University, New York, NY 10027, USA
| | - Hiroo Takayama
- Department of Surgery, Columbia University, New York, NY 10027, USA
| | - Juan B. Grau
- Division of Cardiothoracic Surgery, The Valley Hospital, NJ 07450, USA
- Division of Cardiac Surgery, University of Ottawa Heart Institute, Ottawa, ON K1Y 4W7, Canada
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6
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NADPH Oxidases in Aortic Aneurysms. Antioxidants (Basel) 2022; 11:antiox11091830. [PMID: 36139902 PMCID: PMC9495752 DOI: 10.3390/antiox11091830] [Citation(s) in RCA: 12] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/10/2022] [Revised: 09/08/2022] [Accepted: 09/12/2022] [Indexed: 12/02/2022] Open
Abstract
Abdominal aortic aneurysms (AAAs) are a progressive dilation of the infrarenal aorta and are characterized by inflammatory cell infiltration, smooth muscle cell migration and proliferation, and degradation of the extracellular matrix. Oxidative stress and the production of reactive oxygen species (ROS) have been shown to play roles in inflammatory cell infiltration, and smooth muscle cell migration and apoptosis in AAAs. In this review, we discuss the principles of nicotinamide adenine dinucleotide phosphate oxidase (NADPH oxidase/NOX) signaling and activation. We also discuss the effects of some of the major mediators of NOX signaling in AAAs. Separately, we also discuss the influence of genetic or pharmacologic inhibitors of NADPH oxidases on experimental pre-clinical AAAs. Experimental evidence suggests that NADPH oxidases may be a promising future therapeutic target for developing pharmacologic treatment strategies for halting AAA progression or rupture prevention in the management of clinical AAAs.
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Lypovetska S. ACUTE CORONARY SYNDROME IN A PATIENT WITH MULTIPLY CORONARY ARTERY ECTASIA AND ASCENDING AORTIC ANEURYSM. WIADOMOSCI LEKARSKIE (WARSAW, POLAND : 1960) 2022; 75:1792-1795. [PMID: 35962700 DOI: 10.36740/wlek202207133] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/12/2023]
Abstract
This report describes a case of multiply СAE presenting as a ST-elevation myocardial infarction in an adult patient with bicuspid aortic valve (BAV) and ascending aortic aneurysm. Coronary angiography revealed multiply ectasia in left anterior descending artery and right coronary artery. A computer tomographic aortogram confirmed the presence BAV with signs of calcification and an enlarged ascending aorta. The patient underwent coronary artery bypass grafting of left descending and circumflex coronary arteries and tube graft replacement of ascending aorta in 1 month after ACS in experienced centre. In patients with ACS due to CAE culprit, the emphasis is to restore flow. Medical treatment as well as lifestyle changes and risk factors control, and serial imaging assessment of aortic aneurysm constitute the second part of the management of these patients. We need prospective studies and registries to improve patient care and outcome.
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Affiliation(s)
- Sofiya Lypovetska
- I. HORBACHEVSKY TERNOPIL NATIONAL MEDICAL UNIVERSITY, TERNOPIL, UKRAINE
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8
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Tapasak BE, Malis DJ. The Mystery of Ehlers-Danlos Syndrome: An Autobiographical Case Report. Cureus 2022; 14:e21601. [PMID: 35228959 PMCID: PMC8869278 DOI: 10.7759/cureus.21601] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/25/2022] [Indexed: 11/06/2022] Open
Abstract
Ehlers-Danlos syndrome (EDS) most often presents with the classic symptoms of skin hyperelasticity, hypermobility of joints, atrophic scarring, and fragility of blood vessels. However, EDS can also have uncommon presentations which are much more insidious. This case report details the author’s lifelong experience living with EDS, which was diagnosed after many seemingly unrelated afflictions including fatigue, spontaneous pneumothorax, and gastroesophageal reflux disease. Studies indicate that these complications warrant investigation of the connective tissue disorder with further lifelong follow-up of disease progression. Extra care should be taken to differentiate the disorder from other heritable connective tissue disorders as well as consider the psychosocial issues these patients experience.
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9
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Le Huu A, Olive JK, Cekmecelioglu D, Chatterjee S, Amarasekara HS, Green SY, Coselli JS, Preventza O. Endovascular therapy for patients with heritable thoracic aortic disease. Ann Cardiothorac Surg 2022; 11:31-36. [PMID: 35211383 PMCID: PMC8807421 DOI: 10.21037/acs-2021-taes-109] [Citation(s) in RCA: 10] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/15/2021] [Accepted: 10/04/2021] [Indexed: 02/13/2024]
Abstract
BACKGROUND Patients with genetic or heritable aortic conditions and thoracic aortic aneurysm syndrome often develop cardiovascular abnormalities originating at the aortic root and affecting the entire thoracoabdominal aorta. Although thoracic endovascular aortic repair (TEVAR) is usually avoided in these patients, TEVAR may be worthwhile for those at high risk for surgical complications and in certain emergency circumstances. We explored indications for TEVAR in patients with suspected or confirmed genetic or heritable aortic conditions and investigated early and mid-term outcomes. METHODS Our institutional aortic surgery database was queried for patients with suspected or confirmed Marfan syndrome, Loeys-Dietz syndrome, Ehlers-Danlos syndrome, Turner syndrome, neurofibromatosis, or familial aortic aneurysm and dissection who underwent TEVAR between February 1, 2002 and October 31, 2020. We extracted operative details and in-hospital, follow-up, and survival data. RESULTS Thirty-seven patients who underwent 40 endovascular interventions met the inclusion criteria; 25 previously underwent ascending aorta or aortic root surgery, and 21 previously underwent open thoracoabdominal surgery. Postoperative complications included respiratory failure (24.3%), cardiac complications (16.2%), renal failure (13.5%), tracheostomy (8.1%), and spinal cord ischemia (paraplegia/paraparesis) (8.1%). Follow-up ranged from 1.3 to 8.5 years (median: 3.6 years), with 15 deaths overall (three early/in-hospital). Thirteen patients (35.1%) had 22 repeat interventions (open and endovascular) post-TEVAR; five had the endograft removed. CONCLUSIONS Despite consensus that thoracic aneurysms in patients with genetic or heritable aortic conditions should be treated with conventional open surgery, the outcomes from our study suggest that TEVAR might be suitable in emergency settings or for patients in this population who are not candidates for open surgery, who are at high risk for reintervention, or who have a previously implanted Dacron graft. Nonetheless, lifelong surveillance is important for these patients after TEVAR to monitor for new dissection at distal or proximal landing zones, as repeat interventions are frequent.
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Affiliation(s)
- Alice Le Huu
- Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA
| | - Jacqueline K. Olive
- Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA
| | - Davut Cekmecelioglu
- Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA
- Section of Adult Cardiac Surgery, Department of Cardiovascular Surgery, Texas Heart Institute, Houston, TX, USA
| | - Subhasis Chatterjee
- Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA
- Section of Adult Cardiac Surgery, Department of Cardiovascular Surgery, Texas Heart Institute, Houston, TX, USA
| | - Hiruni S. Amarasekara
- Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA
- Office of Surgical Research, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA
| | - Susan Y. Green
- Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA
- Office of Surgical Research, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA
| | - Joseph S. Coselli
- Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA
- Section of Adult Cardiac Surgery, Department of Cardiovascular Surgery, Texas Heart Institute, Houston, TX, USA
| | - Ourania Preventza
- Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA
- Section of Adult Cardiac Surgery, Department of Cardiovascular Surgery, Texas Heart Institute, Houston, TX, USA
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10
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Rohrer TR, Abuzzahab J, Backeljauw P, Birkegård AC, Blair J, Dahlgren J, Júlíusson PB, Ostrow V, Pietropoli A, Polak M, Romano A, Ross J, Sävendahl L, Miller BS. Long-Term Effectiveness and Safety of Childhood Growth Hormone Treatment in Noonan Syndrome. Horm Res Paediatr 2021; 93:380-395. [PMID: 33440388 DOI: 10.1159/000512429] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/24/2019] [Accepted: 09/25/2020] [Indexed: 11/19/2022] Open
Abstract
INTRODUCTION Few data exist on long-term growth hormone (GH) treatment in patients with Noonan syndrome (NS). OBJECTIVE To evaluate the effectiveness and safety of GH treatment in NS in clinical practice. METHODS Height gain, near-adult height (NAH), and safety were assessed in 2 complementary non-interventional studies: NordiNet® IOS and ANSWER. The safety analysis included 412 patients, and the effectiveness analysis included 84 GH-treated patients (male, n = 67) with ≥4 years' height standard deviation score (HSDS) data. HSDS was determined using national reference (NR) and NS-specific (NSS) data. RESULTS The mean (SD) baseline age was 8.38 (3.57) years; HSDS, -2.76 (1.03); GH dose, 41.6 (11.1) µg/kg/day. The mean (SD) HSDS increase from baseline (ΔHSDS) was 0.49 (0.37) (first year), 0.79 (0.58) (second year), and 1.01 (0.60) (third year) (NR). The mean (SD) HSDS at year 3 was -1.66 (1.00) (NR; 1.06 [1.12] [NSS]). Twenty-four patients achieved NAH. The mean (SD) NAH SDS (NR) was -1.51 (0.60) (154.90 [3.21] cm) in females and -1.79 (1.09) (165.61 [7.19] cm) in males; 70.8% (17/24) had NAH SDS ≥ -2. Adverse drug reactions and GH-unrelated serious adverse events (n = 34) were reported in 22/412 (5.3%) patients. Four neoplasms and 3 cases of scoliosis were reported; no cardiovascular adverse events occurred. CONCLUSIONS GH-treated children with NS achieved substantial height gain during the first 3 years of follow-up. Overall, 24 patients achieved NAH, with 70.8% having NAH SDS ≥ -2. There was no evidence to support a higher prevalence of neoplasm, or cardiac or other comorbidities.
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Affiliation(s)
- Tilman R Rohrer
- Department of Pediatric Endocrinology, University Children's Hospital, Saarland University Medical Center, Homburg, Germany,
| | - Jennifer Abuzzahab
- Diabetes and Endocrine Center, Children's Hospital & Clinics of Minnesota, Saint Paul, Minnesota, USA
| | - Philippe Backeljauw
- Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
| | | | - Joanne Blair
- Department of Paediatric Endocrinology, Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom
| | - Jovanna Dahlgren
- Department of Paediatrics, Region Västra Götaland, Sahlgrenska University Hospital, Gothenburg, Sweden
| | - Pétur Benedikt Júlíusson
- Department of Paediatrics, Haukeland University Hospital, Bergen, Norway.,Department of Health Registry Research and Development, National Institute of Public Health, Bergen, Norway.,Department of Clinical Science, University of Bergen, Bergen, Norway
| | - Vlady Ostrow
- Medical Affairs, Novo Nordisk Inc., Princeton, New Jersey, USA
| | - Alberto Pietropoli
- Global Medical Affairs, Novo Nordisk Health Care AG, Zurich, Switzerland
| | - Michel Polak
- Paediatric Endocrinology, Diabetology and Gynaecology Department, Necker Children's University Hospital, Imagine Institute, Paris, France
| | - Alicia Romano
- Department of Pediatrics, New York Medical College, Valhalla, New York, USA
| | - Judith Ross
- Nemours DuPont Hospital for Children and Department of Pediatrics, Thomas Jefferson University, Philadelphia, Pennsylvania, USA
| | - Lars Sävendahl
- Pediatric Endocrinology, Karolinska University Hospital and Department of Women´s and Children´s Health, Karolinska Institutet, Solna, Sweden
| | - Bradley S Miller
- Pediatric Endocrinology, University of Minnesota Masonic Children's Hospital, Minneapolis, Minnesota, USA
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11
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Jun C, Fang B. Current progress of fluoroquinolones-increased risk of aortic aneurysm and dissection. BMC Cardiovasc Disord 2021; 21:470. [PMID: 34583637 PMCID: PMC8477541 DOI: 10.1186/s12872-021-02258-1] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2021] [Accepted: 09/08/2021] [Indexed: 11/12/2022] Open
Abstract
Aortic aneurysm (AA) and aortic dissection (AD) are major life-threatening diseases around the world. AA is a localized or diffuse dilation of the aorta, while AD is the separation of the layers creating a false lumen within the aortic wall. Fluoroquinolones (FQ) remain one of the most important kind of antibiotics and have a wider clinical use and broad antibacterial spectrum. FQ were also reported to treat infected AA. The most common adverse events (AEs) of FQ are mild and reversible, like headaches, diarrhea and nausea. Due to FQ-related serious AEs, such as tendonitis and tendon rupture, chondrotoxicity, or retinal detachment, QT-prolongation and dysglycemia, the United States Food and Drug Administration (FDA) issued a black box warning for FQ for systemic use in 2016 and updated warnings for FQ several times since then. Of note, in December 2018, FDA issued several "black box warnings" against FQ with the latest safety announcement warning about an increased risk of ruptures in the aorta blood vessel in certain patients. Recently, many studies have indicated an association between FQ and an increase risk of AA and AD. However, the exact mechanism of FQ-induced AA/AD remains unclear. This review aims to highlight the latest research progress of the alarming association between FQ and AA/AD. Moreover, molecular mechanisms of FQ in increasing risk of AA and AD are explored. Hopefully, this review can provide novel insights into FQ-increased the risk of AA/AD and a starting place for stewardship interventions.
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Affiliation(s)
- Cui Jun
- Department of Cardiothoracic Surgery, Xiangyang Central Hospital, Affiliated Hospital of Hubei University of Arts and Science, Xiangyang, 441000, Hubei, China
| | - Bian Fang
- Department of Pharmacy, Featured Preparations of Vitiligo Xiangyang Key Laboratory, Xiangyang Central Hospital, Affiliated Hospital of Hubei University of Arts and Science, Xiangyang, 441000, Hubei, China.
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12
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Harky A, Sokal PA, Hasan K, Papaleontiou A. The Aortic Pathologies: How Far We Understand It and Its Implications on Thoracic Aortic Surgery. Braz J Cardiovasc Surg 2021; 36:535-549. [PMID: 34617429 PMCID: PMC8522328 DOI: 10.21470/1678-9741-2020-0089] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Thoracic aortic diseases contribute to a major part of cardiac surgeries. The severity of pathologies varies significantly from emergency and life-threatening to conservatively managed conditions. Life-threatening conditions include type A aortic dissection and rupture. Aortic aneurysm is an example of a conservatively managed condition. Pathologies that affect the arterial wall can have a profound impact on the presentation of such cases. Several risk factors have been identified that increase the risk of emergency presentations such as connective tissue disease, hypertension, and vasculitis. The understanding of aortic pathologies is essential to improve management and clinical outcomes.
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Affiliation(s)
- Amer Harky
- Department of Cardiothoracic Surgery, Liverpool Heart and Chest Hospital, Liverpool, United Kingdom.,School of Medicine, University of Liverpool, Liverpool, United Kingdom.,Liverpool Centre for Cardiovascular Science, Liverpool Heart and Chest Hospital, Liverpool, United Kingdom
| | | | - Khubbaib Hasan
- School of Medicine, University of Liverpool, Liverpool, United Kingdom
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13
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Evaluating the monogenic contribution and genotype-phenotype correlation in patients with isolated thoracic aortic aneurysm. Eur J Hum Genet 2021; 29:1129-1138. [PMID: 33824467 DOI: 10.1038/s41431-021-00857-2] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/18/2020] [Revised: 02/25/2021] [Accepted: 02/26/2021] [Indexed: 01/09/2023] Open
Abstract
Thoracic aortic aneurysm with or without dissection (TAAD) can be broadly categorized as syndromic TAAD (sTAAD) and isolated TAAD (iTAAD). sTAAD and is highly correlated with genetics. However, although the incidence of iTAAD is much higher, its monogenic contribution is not yet clear. Here, we sequenced 15 known TAAD genes for 578 iTAAD cases from four cardiac centers in China and found that 10.6% patients with a pathogenic/likely pathogenic (P/LP) variant. Other 7.27% of patients carried variants of uncertain significance in these target genes. We further investigated the correlations among genetics, clinical features, and long-term outcomes. Genetic patients showed younger onset ages (P = 1.31E-13) and larger aortic diameter (P = 1.00E-6), with the youngest age in patients with FBN1 P/LP variants. Monogenic variants were also associated with more aortic segments involved (P = 0.043) and complicated with initial dissection (P = 4.50E-5), especially for genetic patients with non-FBN1 P/LP variants. MACEs occurred in 14.9% patients during follow-up of median 55 months. Genetic status (P = 0.001) and initial dissection (P = 3.00E-6) were two major risk factors for poor prognosis. Early onset age was associated with MACEs in non-genetic cases without initial dissection (P = 0.005). Our study revealed the monogenic contribution in known TAAD genes to iTAAD patients. The genotype-phenotype correlations may complement the risk stratification of iTAAD patients and identification of higher risk subgroups, as well as assist the development of tailored precision medicine in iTAAD.
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14
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Odofin X, Houbby N, Hagana A, Nasser I, Ahmed A, Harky A. Thoracic aortic aneurysms in patients with heritable connective tissue disease. J Card Surg 2021; 36:1083-1090. [PMID: 33476431 DOI: 10.1111/jocs.15340] [Citation(s) in RCA: 12] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/12/2020] [Accepted: 12/26/2020] [Indexed: 01/03/2023]
Abstract
BACKGROUND Patients with connective tissue diseases are at high lifetime risk of developing thoracic aortic aneurysms (TAAs) due to defects in extracellular matrix composition which compromise the structural integrity of the aortic wall. It is vital to identify and manage aneurysms early to prevent fatal complications such as dissection or rupture. METHOD This review synthesises information obtained from a thorough literature search regarding the pathophysiology of TAAs in those with heritable connective tissue diseases (HCTDs), the investigations for timely diagnosis and current operative strategies. RESULTS Major complications of open repair (OR) include pneumonia (32%), haemorrhage (31%) and tracheostomy (18%), with a minor risk of vocal cord paresis (9%). For thoracic endovascular aortic repair (TEVAR), high rates of endoleak were documented (38-66.6%). Reintervention rates for TEVAR are also high at 38-44%. Mortality rates were documented as 25% for open repair and vary from 14% to 44% for TEVAR. CONCLUSION OR remains the mainstay of surgical management. While TEVAR use is expanding, it remains the alternative choice due to concerns over endograft durability, limited long-term outcome data and the lack of high-quality evidence regarding its use in HCTD patients.
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Affiliation(s)
- Xuan Odofin
- Faculty of Medicine, Imperial College School of Medicine, Imperial College London, London, UK
| | - Nour Houbby
- Faculty of Medicine, Imperial College School of Medicine, Imperial College London, London, UK
| | - Arwa Hagana
- Faculty of Medicine, Imperial College School of Medicine, Imperial College London, London, UK
| | - Ibrahim Nasser
- Faculty of Medicine, Imperial College School of Medicine, Imperial College London, London, UK.,Leicester Medical School, College of Life Sciences, University of Leicester, Leicester, UK
| | | | - Amer Harky
- Department of Cardiothoracic Surgery, Liverpool Heart and Chest Hospital, Liverpool, UK.,Department of Integrative Biology, Faculty of Life Sciences, University of Liverpool, Liverpool, UK.,Liverpool Centre for Cardiovascular Science, Liverpool Heart and Chest Hospital, Liverpool, UK
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15
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Harky A, Hussain SMA, MacCarthy-Ofosu B, Ahmad MU. The Role of Thoracic Endovascular Aortic Repair (TEVAR) of Thoracic Aortic Diseases in Patients with Connective Tissue Disorders - A Literature Review. Braz J Cardiovasc Surg 2020; 35:977-985. [PMID: 33306324 PMCID: PMC7731863 DOI: 10.21470/1678-9741-2019-0367] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022] Open
Abstract
OBJECTIVE To review the currently available literature to define the role of thoracic endovascular aortic repair (TEVAR) in patients with connective tissue disorders (CTD). METHODS A comprehensive electronic database search was performed in PubMed, SCOPUS, Embase, Google scholar, and OVID to identify all the articles that reported on outcomes of utilizing TEVAR in patients with CTD during elective and emergency settings. The search was not limited to time or language of the published study. RESULTS All the relevant studies have been summarized in its correspondence section. The outcomes were analyzed in narrative format. The role of TEVAR has been elaborated as per each study. Currently, there is limited large cohort size studies outlining the use of TEVAR in patients with CTD. The use of endovascular repair in patients with CTD is limited due to progressive aortic dilatations and high possibility of further reinterventions at later stage of life. CONCLUSION Open repair remains the gold standard method of intervention in young patients with progressive CTD, especially in the setting of acute type A aortic dissection. However, TEVAR can be sought as a reliable alternative in emergency setting of diseases involving the descending thoracic aorta; yet the long-term data needs to be published to support such practice.
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Affiliation(s)
- Amer Harky
- Department of Cardiothoracic Surgery, Liverpool Heart and Chest, Liverpool, UK
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16
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Tanga CF, Fakhoury E, Ham PB, Dosluoglu HH, Harris LM. Ruptured abdominal aortic aneurysm in an 11-year-old with multiple peripheral artery aneurysms. JOURNAL OF VASCULAR SURGERY CASES INNOVATIONS AND TECHNIQUES 2020; 6:539-542. [PMID: 33134637 PMCID: PMC7588751 DOI: 10.1016/j.jvscit.2020.07.015] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 05/06/2020] [Accepted: 07/23/2020] [Indexed: 11/25/2022]
Abstract
Pediatric abdominal aortic aneurysms (AAAs) are rarely encountered in clinical practice. The combination of a pediatric AAA in a patient with multiple peripheral artery aneurysms is even more rare. We report the management of an 11-year-old boy who presented with a ruptured AAA who also had multiple peripheral arterial aneurysms. Infectious, genetic, and inflammatory workup was negative, classifying this aneurysm as congenital.
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Affiliation(s)
| | - Elias Fakhoury
- Department of Vascular Surgery, University at Buffalo, Buffalo, NY
| | - P Benson Ham
- Department of Vascular Surgery, University at Buffalo, Buffalo, NY
| | | | - Linda M Harris
- Department of Vascular Surgery, University at Buffalo, Buffalo, NY
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17
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Blackstock CD, Jackson BM. Open Surgical Repair of Abdominal Aortic Aneurysms Maintains a Pivotal Role in the Endovascular Era. Semin Intervent Radiol 2020; 37:346-355. [PMID: 33041480 DOI: 10.1055/s-0040-1715881] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022]
Abstract
Since the advent of endovascular aortic repair (EVAR) nearly three decades ago, there has been a paradigm shift in the treatment of the abdominal aortic aneurysm (AAA) to favor EVAR due to its reduced operative mortality, less invasive nature, and faster recovery times. However, more recently there has been an accumulation of data from large meta-analyses and randomized clinical trials revealing that EVAR has no survival benefit after approximately 2 years and is associated with substantially higher rates of reintervention and aneurysm rupture in the long term. These findings call into question the durability of EVAR compared with open aortic repair and emphasize the need for surgeons to remain competent with open aortic surgery in the modern era. This article will provide comprehensive review of a large body of literature comparing endovascular repair to open aortic surgery for the management of AAAs, and it will offer an overview of the open surgical repair technique for AAAs.
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Affiliation(s)
- Christopher D Blackstock
- Division of Vascular Surgery and Endovascular Therapy, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Benjamin M Jackson
- Division of Vascular Surgery and Endovascular Therapy, University of Pennsylvania, Philadelphia, Pennsylvania
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18
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Pala R, Anju VT, Dyavaiah M, Busi S, Nauli SM. Nanoparticle-Mediated Drug Delivery for the Treatment of Cardiovascular Diseases. Int J Nanomedicine 2020; 15:3741-3769. [PMID: 32547026 PMCID: PMC7266400 DOI: 10.2147/ijn.s250872] [Citation(s) in RCA: 88] [Impact Index Per Article: 17.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022] Open
Abstract
Cardiovascular diseases (CVDs) are one of the foremost causes of high morbidity and mortality globally. Preventive, diagnostic, and treatment measures available for CVDs are not very useful, which demands promising alternative methods. Nanoscience and nanotechnology open a new window in the area of CVDs with an opportunity to achieve effective treatment, better prognosis, and less adverse effects on non-target tissues. The application of nanoparticles and nanocarriers in the area of cardiology has gathered much attention due to the properties such as passive and active targeting to the cardiac tissues, improved target specificity, and sensitivity. It has reported that more than 50% of CVDs can be treated effectively through the use of nanotechnology. The main goal of this review is to explore the recent advancements in nanoparticle-based cardiovascular drug carriers. This review also summarizes the difficulties associated with the conventional treatment modalities in comparison to the nanomedicine for CVDs.
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Affiliation(s)
- Rajasekharreddy Pala
- Department of Biomedical and Pharmaceutical Sciences, Harry and Diane Rinker Health Science Campus, Chapman University, Irvine, CA92618, USA
- Department of Medicine, University of California Irvine, Irvine, CA92868, USA
| | - V T Anju
- Department of Biochemistry and Molecular Biology, School of Life Sciences, Pondicherry University, Puducherry, India
| | - Madhu Dyavaiah
- Department of Biochemistry and Molecular Biology, School of Life Sciences, Pondicherry University, Puducherry, India
| | - Siddhardha Busi
- Department of Microbiology, School of Life Sciences, Pondicherry University, Puducherry, India
| | - Surya M Nauli
- Department of Biomedical and Pharmaceutical Sciences, Harry and Diane Rinker Health Science Campus, Chapman University, Irvine, CA92618, USA
- Department of Medicine, University of California Irvine, Irvine, CA92868, USA
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19
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Manda G, Chaziya P, Mwafulirwa W, Kasenda S, Borgstein E. Successful open surgical repair of an infrarenal, abdominal aortic aneurysm (AAA) in a young Malawian female: A case report. Malawi Med J 2020; 31:256-258. [PMID: 32128036 PMCID: PMC7036426 DOI: 10.4314/mmj.v31i4.7] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
Affiliation(s)
| | | | | | | | - Eric Borgstein
- Queen Elizabeth Central Hospital, Blantyre, Malawi.,University of Malawi, College of Medicine, Blantyre, Malawi
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20
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Hamandi M, Bolin ML, Fan J, Lanfear AT, Woolbert SK, Baxter RD, DiMaio JM, Brinkman WT. A Newly Discovered Genetic Disorder Associated With Life-Threatening Aortic Disease in a 6-Year-Old Boy. J Investig Med High Impact Case Rep 2020; 8:2324709620909234. [PMID: 32102558 PMCID: PMC7047236 DOI: 10.1177/2324709620909234] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Abstract
Aortic aneurysms in children are rare and when present are usually caused by a connective tissue disorder. In this article, we present a case of multiple aortic aneurysms in an adolescent with a novel finding of a gene variation that is associated with aortic disease.
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Affiliation(s)
| | | | - Joy Fan
- Baylor Scott and White-The Heart Hospital, Plano, TX, USA
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21
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Goel N, Jain D, Savlania A, Bansal A. Thoracoabdominal Aortic Aneurysm Repair: What Should the Anaesthetist Know? Turk J Anaesthesiol Reanim 2019; 47:1-11. [PMID: 31276105 DOI: 10.5152/tjar.2018.39129] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/08/2018] [Accepted: 08/06/2018] [Indexed: 11/22/2022] Open
Abstract
The surgical repair of descending thoracic aortic (DTA) and thoracoabdominal aortic aneurysms (TAAAs) presents one of the greatest challenges for anaesthesiologists. The challenge comes from the fine balance of complex medical issues in the setting of altered physiology that occurs during the perioperative period. Patients presenting for TAAA repair usually have multiple pre-existing comorbid conditions involving their cardiac, pulmonary and renal systems; and aneurysm repair poses a direct and immediate threat to these systems in addition to that to the gastrointestinal and neurologic systems. Operative mortality in thoracoabdominal aortic surgery is quite high to the extent of 5%-12% with a 5-year survival rate of 70%-79% for DTA aneurysm and 59% for thoracoabdominal aortic aneurysm surgeries. Complex haemodynamic changes associated with the clamping and declamping of aorta requires thorough knowledge and expertise for the management of TAAA. We present a brief review on the anaesthetic management and possible complications that anaesthetists should be aware of during TAAA repair.
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Affiliation(s)
- Nitika Goel
- Department of Anaesthesia, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Divya Jain
- Department of Anaesthesia, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Ajay Savlania
- Department of Vascular Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Ashwani Bansal
- Department of Cardiothoracic and Vascular Surgery, Max Hospital, Chandigarh, India
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22
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Manda GE, Chaziya P, Mwafulirwa W, Kasenda S, Borgstein E. Successful open surgical repair of an infrarenal, abdominal aortic aneurysm (AAA) in a young Malawian female: A case report. Malawi Med J 2019; 30:215-217. [PMID: 30627359 PMCID: PMC6307054 DOI: 10.4314/mmj.v30i3.15] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
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23
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Wang Y, Wang H, Xu N. Multiple intracranial aneurysms and abdominal aortic occlusion in a young woman: A case report. Medicine (Baltimore) 2018; 97:e10934. [PMID: 29879036 PMCID: PMC5999449 DOI: 10.1097/md.0000000000010934] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
RATIONALE Multiple intracranial aneurysms occur in 10% to 30% patients with cerebral aneurysms. PATIENT CONCERNS We reported a case of multiple intracranial aneurysms concurrent with abdominal aortic occlusion (AAO) in a 29-year-old woman was admitted because of abrupt onset of severe headache, vomiting, and dizziness for 26 hours. She complained sudden onset of severe headache, vomiting, and dizziness. DIAGNOSES Head computed tomography (CT) angiogram revealed 2 aneurysms of the anterior communicating artery (ACA) and a third aneurysm at the right middle cerebral artery (MCA). A diagnosis of multiple intracranial aneurysms concurrent with abdominal aortic occlusion (AAO) was made. INTERVENTIONS We clipped the 2 aneurysms at the ACA via a right pterional approach. The transfemoral approach failed because of an unsuspected AAO. A right carotid artery approach was then employed to embolize the aneurysm at the right MCA with three coils. OUTCOMES Magnetic resonance angiography (MRA) at 7 days after the embolization demonstrated complete disappearance of all the intracranial aneurysms, but AAO was still present. The patient remained asymptomatic during 5-years of follow-up. LESSONS The case highlights the importance of a thorough physical examination, and in rare cases, AAO or other abdominal aortic abnormalities should be considered in young nonsmoking females. Successful treatment can be achieved by aneurysm clipping and embolization.
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24
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Abstract
A variety of syndromes are associated with thoracoabdominal aortic pathologies. While these diseases are collectively rare, the presence of advanced or unusual aortic disease at a young age should raise suspicion of an underlying syndrome. Similarly, patients with a known syndrome require close monitoring in anticipation of future aortic disease. In this article, the syndromes most commonly encountered in clinical practice are reviewed, including Marfan syndrome (MFS) and other connective tissue disorders, Turner syndrome (TS), autosomal dominant polycystic kidney disease (ADPKD), neurofibromatosis (NF), Williams syndrome (WS), Alagille syndrome (AGS), and DiGeorge syndrome (DGS). The distinct clinical, imaging, and management features of each disorder are discussed. Attention is focused on the unique patterns of aortic disease in each syndrome, emphasizing the role of recent imaging modalities and treatment strategies. Ancillary and distinguishing aspects of the syndromes that aid in diagnosis are also highlighted.
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Affiliation(s)
- Evan J Zucker
- Department of Radiology, Stanford University School of Medicine, Stanford, CA, USA
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25
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Kiru G, Bicknell C, Falaschetti E, Powell J, Poulter N. An evaluation of the effect of an angiotensin-converting enzyme inhibitor on the growth rate of small abdominal aortic aneurysms: a randomised placebo-controlled trial (AARDVARK). Health Technol Assess 2018; 20:1-180. [PMID: 27488944 DOI: 10.3310/hta20590] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
Abstract
BACKGROUND Although data are inconsistent, angiotensin-converting enzyme inhibitors (ACE-Is) have been associated with a reduced incidence of abdominal aortic aneurysm (AAA) rupture in analysis of administrative databases. OBJECTIVES (1) To investigate whether or not the ACE-I perindopril (Coversyl arginine, Servier) reduces small AAA growth rate and (2) to evaluate blood pressure (BP)-independent effects of perindopril on small AAA growth and to compare the repeatability of measurement of internal and external aneurysm diameters. DESIGN A three-arm, multicentre, single-blind, randomised placebo-controlled trial. SETTING Fourteen hospitals in England. PARTICIPANTS Men or women aged ≥ 55 years with an AAA of 3.0-5.4 cm in diameter by internal or external measurement according to ultrasonography and who met the trial eligibility criteria. INTERVENTIONS Patients were randomised to receive 10 mg of perindopril arginine daily, 5 mg of the calcium channel blocker amlodipine daily or placebo daily. MAIN OUTCOME MEASURES The primary outcome was AAA diameter growth using external measurements in the longitudinal plane, which in-trial studies suggested was the preferred measure. Secondary outcome measures included AAA rupture, AAA repair, modelling of the time taken for the AAA to reach the threshold for intervention (5.5 cm) or referral for surgery, tolerance of study medication (measured by compliance, adverse events and quality of life) and a comparison of the repeatability of measures of internal and external AAA diameter. Patients were followed up every 3-6 months over 2 years. RESULTS In total, 227 patients were recruited and randomised into the three groups, which were generally well matched at baseline. Multilevel modelling was used to determine the maximum likelihood estimates for AAA diameter growth. No significant differences in the estimates of annual growth were apparent [1.68 (standard error 0.02) mm, 1.77 (0.02) mm and 1.81 (0.02) mm in the placebo, perindopril and amlodipine groups, respectively]. Similarly, no significant differences in the slopes of modelled growth over time were apparent between perindopril and placebo (p = 0.78) or between perindopril and amlodipine (p = 0.89). The results were essentially unaffected by adjustment for potential confounders. Compliance, measured by pill counts, was good throughout (> 80% at all visit time points). There were no significant in-trial safety concerns. Six patients withdrew because of adverse events attributed to the study medications (n = 2 perindopril, n = 4 amlodipine). No patients ruptured their AAA and 27 underwent elective surgery during the trial (n = 9 placebo, n = 10 perindopril, n = 8 amlodipine). CONCLUSIONS We were unable to demonstrate a significant impact of perindopril compared with placebo or amlodipine on small AAA growth over a 2-year period. Furthermore, there were no differences in the times to reach a diameter of 5.5 cm or undergo surgery among the three groups. Perindopril and amlodipine were well tolerated by this population. External AAA measurements were found to be more repeatable than internal measurements. The observed AAA growth measurement variability was greater than that expected pre trial. This, combined with slower than expected mean growth rates, resulted in our having limited power to detect small differences between growth rates and hence this adds uncertainty to the interpretation of the results. Several further analyses are planned including a multivariate analysis of determinants of AAA growth, an evaluation of the possible differential effect of perindopril on fast AAA growth and an investigation into the roles of central BP and BP variability on AAA growth. TRIAL REGISTRATION Current Controlled Trials ISRCTN51383267. FUNDING This project was funded by the National Institute for Health Research (NIHR) Health Technology Assessment programme and will be published in full in Health Technology Assessment; Vol. 20, No. 59. See the NIHR Journals Library website for further project information. The NIHR Biomedical Research Centre based at Imperial College NHS Trust supported the trial. Servier provided perindopril at no charge.
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Affiliation(s)
- Gaia Kiru
- Imperial Clinical Trials Unit, School of Public Health, Imperial College London, London, UK
| | - Colin Bicknell
- Department of Surgery and Cancer, Imperial College London, London, UK
| | - Emanuela Falaschetti
- Imperial Clinical Trials Unit, School of Public Health, Imperial College London, London, UK
| | - Janet Powell
- Department of Surgery and Cancer, Imperial College London, London, UK
| | - Neil Poulter
- Imperial Clinical Trials Unit, School of Public Health, Imperial College London, London, UK
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26
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Krepp JM, Roman MJ, Devereux RB, Bruce A, Prakash SK, Morris SA, Milewicz DM, Holmes KW, Ravekes W, Shohet RV, Pyeritz RE, Maslen CL, Kroner BL, Eagle KA, Preiss L, Asch FM. Bicuspid and unicuspid aortic valves: Different phenotypes of the same disease? Insight from the GenTAC Registry. CONGENIT HEART DIS 2017; 12:740-745. [PMID: 28805011 PMCID: PMC5819742 DOI: 10.1111/chd.12520] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/16/2017] [Revised: 06/17/2017] [Accepted: 06/26/2017] [Indexed: 01/26/2023]
Abstract
BACKGROUND Unicuspid aortic valve (UAV) is a rare disorder, often difficult to distinguish from bicuspid aortic valve (BAV). BAV and UAV share valve pathology such as the presence of a raphe, leaflet fusion, aortic stenosis, aortic regurgitation, and/or ascending aortic dilatation, but a comprehensive echocardiographic comparison of patients with UAV and BAV has not been previously performed. METHODS We investigated UAV and BAV patients at an early stage of disease included in GenTAC, a national registry of genetically related aortic aneurysms and associated cardiac conditions. Clinical and echocardiographic data from the GenTAC Registry were compared between 17 patients with UAV and 17 matched-controls with BAV. RESULTS Baseline characteristics including demographics, clinical findings including family history of BAV and aortic aneurysm/coarctation, and echocardiographic variables were similar between BAV and UAV patients; aortic stenosis was more common and more severe in patients with UAV. This was evidenced by higher mean and peak gradient, smaller aortic valve area, and more advanced valvular degeneration (all P < .05). There were no significant differences in aortic dimensions, with a similar pattern of enlargement of the ascending aorta. CONCLUSIONS The similar baseline characteristics with more accelerated aortic valve degeneration and stenosis suggest that UAV represents an extreme in the spectrum of BAV syndromes. Therefore, it is reasonable to consider application of recommendations for the management of patients with BAV to those with the rarer UAV.
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27
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Guevara C, Farias G, Bulatova K, Alarcón P, Soruco W, Robles C, Morales M. NOTCH 1 Mutation in a Patient with Spontaneous and Recurrent Dissections of Extracranial Arteries. Front Neurol 2017. [PMID: 28649221 PMCID: PMC5465274 DOI: 10.3389/fneur.2017.00245] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/04/2023] Open
Abstract
Dissections of extracranial arteries are estimated to account for only 2% of all ischemic strokes but for approximately 20% of strokes in patients younger than 45 years old. Most dissections of extracranial arteries involve some trauma stretch, mechanical stress, or connective tissue abnormalities. In the absence of these disorders, determining the etiology of recurrent extracranial dissections is quite challenging because the underlying nature of these cases is poorly understood. We report the case of a 44-year-old female with recurrent dissections of the vertebral and carotid arteries associated with a heterozygous mutation p.Pro2122Leu in the NOTCH 1 gene. Her mother with a thoracic aortic aneurysm was also positive for this variant.
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Affiliation(s)
- Carlos Guevara
- Clínica de Neurología, Servicio de Neurología y Neurocirugía, Hospital Clínico Universidad de Chile, Santiago, Chile
| | - Gonzalo Farias
- Clínica de Neurología, Servicio de Neurología y Neurocirugía, Hospital Clínico Universidad de Chile, Santiago, Chile
| | - Kateryna Bulatova
- Clínica de Neurología, Servicio de Neurología y Neurocirugía, Hospital Clínico Universidad de Chile, Santiago, Chile
| | - Pablo Alarcón
- Sección Genética, Departamento de Medicina, Hospital Clínico Universidad de Chile, Santiago, Chile
| | - Wendy Soruco
- Clínica de Neurología, Servicio de Neurología y Neurocirugía, Hospital Clínico Universidad de Chile, Santiago, Chile
| | - Carlos Robles
- Sección Neurorradiologia, Servicio de Imágenes, Hospital Clínico Universidad de Chile, Santiago, Chile
| | - Marcelo Morales
- Clínica de Cardiologia, Hospital San Juan de Dios, Santiago, Chile
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28
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Venkatesh P, Phillippi J, Chukkapalli S, Rivera-Kweh M, Velsko I, Gleason T, VanRyzin P, Aalaei-Andabili SH, Ghanta RK, Beaver T, Chan EKL, Kesavalu L. Aneurysm-Specific miR-221 and miR-146a Participates in Human Thoracic and Abdominal Aortic Aneurysms. Int J Mol Sci 2017; 18:ijms18040875. [PMID: 28425970 PMCID: PMC5412456 DOI: 10.3390/ijms18040875] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/16/2017] [Revised: 04/10/2017] [Accepted: 04/11/2017] [Indexed: 01/02/2023] Open
Abstract
Altered microRNA expression is implicated in cardiovascular diseases. Our objective was to determine microRNA signatures in thoracic aortic aneurysms (TAAs) and abdominal aortic aneurysms (AAAs) compared with control non-aneurysmal aortic specimens. We evaluated the expression of fifteen selected microRNA in human TAA and AAA operative specimens compared to controls. We observed significant upregulation of miR-221 and downregulation of miR-1 and -133 in TAA specimens. In contrast, upregulation of miR-146a and downregulation of miR-145 and -331-3p were found only for AAA specimens. Upregulation of miR-126 and -486-5p and downregulation of miR-30c-2*, -155, and -204 were observed in specimens of TAAs and AAAs. The data reveal microRNA expression signatures unique to aneurysm location and common to both thoracic and abdominal pathologies. Thus, changes in miR-1, -29a, -133a, and -221 are involved in TAAs and miR-145, -146, and -331-3p impact AAAs. This work validates prior studies on microRNA expression in aneurysmal diseases.
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Affiliation(s)
| | - Julie Phillippi
- Department of Cardiothoracic Surgery, University of Pittsburgh School of Medicine, Pittsburgh, PA 15219, USA.
| | - Sasanka Chukkapalli
- Department of Periodontology, University of Florida, Gainesville, FL 32610, USA.
| | - Mercedes Rivera-Kweh
- Department of Periodontology, University of Florida, Gainesville, FL 32610, USA.
| | - Irina Velsko
- Department of Periodontology, University of Florida, Gainesville, FL 32610, USA.
| | - Thomas Gleason
- Department of Cardiothoracic Surgery, University of Pittsburgh School of Medicine, Pittsburgh, PA 15219, USA.
| | - Paul VanRyzin
- Department of Cardiothoracic Surgery, University of Pittsburgh School of Medicine, Pittsburgh, PA 15219, USA.
| | | | - Ravi Kiran Ghanta
- Michael E. Debakey Department of Surgery, Baylor College of Medicine, Houston, TX 77030, USA.
| | - Thomas Beaver
- Departments of Surgery, University of Florida, Gainesville, FL 32610, USA.
| | | | - Lakshmyya Kesavalu
- Department of Periodontology, University of Florida, Gainesville, FL 32610, USA.
- Department of Oral Biology, University of Florida, Gainesville, FL 32610, USA.
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29
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Love B. Neural Interventions. Biomaterials 2017. [DOI: 10.1016/b978-0-12-809478-5.00012-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
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Salve GG, Javali SR, Dalvi BV, Krishnanaik S. Modified pediatric Bentall procedure: A novel technique in a rare case. Ann Pediatr Cardiol 2016; 9:244-7. [PMID: 27625523 PMCID: PMC5007934 DOI: 10.4103/0974-2069.189124] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/24/2022] Open
Abstract
Aneurysms of ascending aorta are rarely seen in pediatric age group. Only few cases with Marfans syndrome have been reported in the literature. Preferred treatment for these children has been the standard Bentall procedure (aortic root replacement with composite graft prosthesis). We report a 4-year-old male child with huge aneurysm of ascending aorta and aortic root dilation with severe aortic regurgitation, having phenotypic features of Loeys-Dietz syndrome type I. He underwent Bentall procedure with a novel modification (medial trap-door technique for coronary reimplantation). Short-term result of this procedure is encouraging and he is asymptomatic for the last 14 months of follow-up.
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Affiliation(s)
- Gananjay G Salve
- Department of Pediatric Cardiovascular and Thoracic Surgery, Seven Hills Hospital, Marol-Maroshi Road, Andheri East, Mumbai, India
| | - Satish R Javali
- Department of Pediatric Cardiovascular and Thoracic Surgery, Seven Hills Hospital, Marol-Maroshi Road, Andheri East, Mumbai, India
| | - Bharat V Dalvi
- Department of Pediatric Cardiovascular and Thoracic Surgery, Seven Hills Hospital, Marol-Maroshi Road, Andheri East, Mumbai, India
| | - Shivaprakash Krishnanaik
- Department of Pediatric Cardiovascular and Thoracic Surgery, Seven Hills Hospital, Marol-Maroshi Road, Andheri East, Mumbai, India
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31
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Versnaeyen H, Saey V, Vermeiren D, Chiers K, Ducatelle R. Intermittent Haemoptysis due to an Aortobronchial Fistula in a Warmblood Mare. J Comp Pathol 2016; 155:213-217. [PMID: 27535296 DOI: 10.1016/j.jcpa.2016.07.007] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/30/2016] [Revised: 07/03/2016] [Accepted: 07/12/2016] [Indexed: 10/21/2022]
Abstract
A 7-year-old warmblood mare showed sudden onset of mild intermittent haemoptysis. Clinical examination revealed no significant abnormalities. Haematological examination showed mild anaemia, hypoalbuminaemia and neutrophilia. Coagulation tests were normal. Endoscopic examination revealed unilateral pulmonary haemorrhage with blood clots in the bronchi and trachea. Treatment with antibiotics was started and the horse was given stable rest. Two weeks later, the horse was found dead with blood and frothy sputum leaking from the nostrils. Post-mortem examination revealed a large thoracic aortic aneurysm communicating with a pseudoaneurysm that had formed a fistula into a right bronchial branch. Microscopical examination of the aneurysm showed extensive medial fibrosis with prominent degeneration, fragmentation and mineralization of the elastic fibres and deposition of mucoid material in the tunica media. The pseudoaneurysm was lined by collagen bundles admixed with fibroblasts and a small amount of adipose tissue. Aortobronchial fistula is a rare condition in man that is usually associated with primary aortic pathology, most often aneurysms. To the authors' knowledge this is the first case of a fatal aortobronchial fistula in a horse or any other animal species.
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Affiliation(s)
- H Versnaeyen
- Department of Veterinary Pathology, Faculty of Veterinary Medicine, University of Ghent, Belgium.
| | - V Saey
- Department of Veterinary Pathology, Faculty of Veterinary Medicine, University of Ghent, Belgium
| | - D Vermeiren
- Veterinary Practice, Perbeemd, Sint-Lenaartseweg 67, Loenhout, Belgium
| | - K Chiers
- Department of Veterinary Pathology, Faculty of Veterinary Medicine, University of Ghent, Belgium
| | - R Ducatelle
- Department of Veterinary Pathology, Faculty of Veterinary Medicine, University of Ghent, Belgium
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33
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Vascular manifestations of syndromic aortopathies: role of current and emerging imaging techniques. Clin Radiol 2015; 70:1344-54. [DOI: 10.1016/j.crad.2015.08.008] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/08/2014] [Revised: 08/10/2015] [Accepted: 08/12/2015] [Indexed: 02/07/2023]
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34
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Chahwala V, Tashiro J, Baqai A, Gologorsky E, Rey J, Robinson HR. Endovascular repair of a thoracic aortic aneurysm in pregnancy at 22 weeks of gestation. J Vasc Surg 2015; 62:1323-5. [DOI: 10.1016/j.jvs.2014.04.037] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/03/2014] [Accepted: 04/10/2014] [Indexed: 11/15/2022]
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Thoracic aorta aneurysm successfully operated on during pregnancy. POLISH JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY 2015; 11:321-3. [PMID: 26336442 PMCID: PMC4283891 DOI: 10.5114/kitp.2014.45684] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 06/23/2013] [Revised: 06/29/2013] [Accepted: 09/16/2013] [Indexed: 11/17/2022]
Abstract
We present the case of an asymptomatic 26-year-old female patient with a huge thoracic aneurysm discovered at a routine echo screening. The patient had previously been operated on for coarctation of the aorta in childhood and also had diagnosed bicuspid aortic valve. The operation was carried out in extracorporeal circulation at the 22nd week of gestation without any complications. A few months later in the 38th week of pregnancy a baby girl was delivered by cesarean section with an Apgar score of 10. The patient is scheduled for implantation of a stent graft to the descending aorta, because the CT of the chest done a few months after delivery confirmed presence of a significant aneurysm located just below the left subclavian artery.
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Mudoni A, Cornacchiari M, Gallieni M, Guastoni C, McGrogan D, Logias F, Ferramosca E, Mereghetti M, Inston N. Aneurysms and pseudoaneurysms in dialysis access. Clin Kidney J 2015; 8:363-7. [PMID: 26251700 PMCID: PMC4515897 DOI: 10.1093/ckj/sfv042] [Citation(s) in RCA: 60] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/14/2015] [Accepted: 04/28/2015] [Indexed: 11/14/2022] Open
Abstract
Aneurysms are a common and often difficult complication seen with arteriovenous vascular access for haemodialysis. The purpose of this narrative review is to define and describe the scale of the problem and suggested therapeutic strategies. A narrative review of the published literature illustrated by individual cases is presented with the aim of summarising the relevant literature. The definitions of aneurysm are inconsistent throughout the literature and therefore systematic review is impossible. They vary from qualitative descriptions to quantitative definitions using absolute size, relative size and also size plus characteristics. The incidence and aetiology are also ill defined but separation into true aneurysms and false, or pseudoaneurysms may be helpful in planning treatment, which may be conservative, surgical or radiological. The lack of useful definitions and classification along with the multitude of management strategies proposed make firm evidence based conclusions difficult to draw. Further robust well designed studies are required to define best practice for this common problem.
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Affiliation(s)
- Anna Mudoni
- Nephrology and Dialysis Unit , Cardinale G. Panico Hospital , Tricase , LE , Italy
| | | | - Maurizio Gallieni
- Nephrology and Dialysis Unit , San Carlo Borromeo Hospital , Milano , Italy ; Department of Clinical and Biomedical Sciences 'Luigi Sacco' , University of Milano , Milano , Italy
| | - Carlo Guastoni
- Nephrology Unit , Magenta Hospital , Magenta, Milan , Italy
| | - Damian McGrogan
- Department of Renal Surgery and Nephrology , Queen Elizabeth Hospital Birmingham, University Hospital Birmingham NHS Trust , Birmingham , UK
| | - Francesco Logias
- Network of Nephrology and Dialysis , ASL , Sorgono, Nuoro , Italy
| | - Emiliana Ferramosca
- Nephrology, Dialysis and Hypertension , S. Orsola-Malpighi University Hospital , Bologna , Italy
| | | | - Nicholas Inston
- Department of Renal Surgery and Nephrology , Queen Elizabeth Hospital Birmingham, University Hospital Birmingham NHS Trust , Birmingham , UK
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Saade C, Pandya B, Raza M, Meghani M, Asti D, Ghavami F. 9.1 cm abdominal aortic aneurysm in a 69-year-old male patient. World J Cardiol 2015; 7:157-160. [PMID: 25810816 PMCID: PMC4365305 DOI: 10.4330/wjc.v7.i3.157] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/01/2014] [Revised: 12/07/2014] [Accepted: 01/20/2015] [Indexed: 02/06/2023] Open
Abstract
We are presenting a case of one of the largest un-ruptured abdominal aortic aneurysm ever reported. Presented here is a rare case of a 69-year-old active smoker male with history of hypertension and incidental diagnosis of abdominal aortic aneurysm of 6.2 cm in 2003, who refused surgical intervention at the time of diagnosis with continued smoking habit and was managed medically. Patient was subsequently admitted in 2012 to the hospital due to unresponsiveness secondary to hypoglycemia along with diagnosis of massive symptomatic pulmonary embolism and non-ST elevation myocardial infarction. With the further inpatient workup along with known history of abdominal aortic aneurysm, subsequent computed tomography scan of abdomen pelvis revealed increased in size of infrarenal abdominal aortic aneurysm to 9.1 cm of without any signs of rupture. Patient was unable to undergo any surgical intervention this time because of his medical instability and was eventually passed away under hospice care.
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39
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Giordano BP, Tuli SY, Tuli SS. An uncommon cause for a preschool child's chronic cough. J Pediatr Health Care 2014; 28:267-71. [PMID: 24361051 DOI: 10.1016/j.pedhc.2013.10.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/15/2013] [Revised: 10/30/2013] [Accepted: 10/30/2013] [Indexed: 11/28/2022]
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Emergent endovascular vs. open surgery repair for ruptured abdominal aortic aneurysms: a meta-analysis. PLoS One 2014; 9:e87465. [PMID: 24498112 PMCID: PMC3909181 DOI: 10.1371/journal.pone.0087465] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/07/2013] [Accepted: 12/27/2013] [Indexed: 12/13/2022] Open
Abstract
Objectives To systematically review studies comparing peri-operative mortality and length of hospital stay in patients with ruptured abdominal aortic aneurysms (rAAAs) who underwent endovascular aneurysm repair (EVAR) to patients who underwent open surgical repair (OSR). Methods The Medline, Cochrane, EMBASE, and Google Scholar databases were searched until Apr 30, 2013 using keywords such as abdominal aortic aneurysm, emergent, emergency, rupture, leaking, acute, endovascular, stent, graft, and endoscopic. The primary outcome was peri-operative mortality and the secondary outcome was length of hospital stay. Results A total of 18 studies (2 randomized controlled trials, 5 prospective studies, and 11 retrospective studies) with a total of 135,734 rAAA patients were included. rAAA patients who underwent EVAR had significantly lower peri-operative mortality compared to those who underwent OSR (overall OR = 0.62, 95% CI = 0.58 to 0.67, P<0.001). rAAA patients with EVAR also had a significantly shorter mean length of hospital stay compared to those with OSR (difference in mean length of stay ranged from −2.00 to −19.10 days, with the overall estimate being −5.25 days (95% CI = −9.23 to −1.26, P = 0.010). There was no publication bias and sensitivity analysis showed good reliability. Conclusions EVAR confers significant benefits in terms of peri-operative mortality and length of hospital stay. There is a need for more randomized controlled trials to compare outcomes of EVAR and OSR for rAAA.
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Durdu T, Yilmaz F, Sönmez BM, Ulgen S, Demir A, Yılmaz MS, Arslan ED, Hakbilir O. An unusual presentation of ruptured abdominal aorta aneurysm. AMERICAN JOURNAL OF CASE REPORTS 2013; 14:267-9. [PMID: 23900449 PMCID: PMC3726238 DOI: 10.12659/ajcr.889271] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/09/2013] [Accepted: 05/17/2013] [Indexed: 11/27/2022]
Abstract
Patient: Female, 65 Final Diagnosis: Ruptured abdominal aorta aneursym Symptoms: — Medication: — Clinical Procedure: After surgery the patient was discharged without sequelae Specialty: Surgery
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Affiliation(s)
- Tamer Durdu
- Emergency Department, Ankara Numune Training and Research Hospital, Ankara, Turkey
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Interrupted aortic arch in an adult with polycystic kidney disease. Case Rep Med 2013; 2013:404710. [PMID: 23840219 PMCID: PMC3690743 DOI: 10.1155/2013/404710] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/02/2013] [Revised: 05/08/2013] [Accepted: 05/20/2013] [Indexed: 11/21/2022] Open
Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease and is responsible for 8–10% of patients with end-stage renal failure. The major extrarenal complications of ADPKD are cardiovascular abnormalities. Interrupted aortic arch (IAA) is a lethal congenital cardiac abnormality seen with a frequency of 3/1000000 births and is defined as a segment of the arcus aorta being atresic. In the literature, there are no any reports showing that polycystic kidney disease and interrupted aortic arch occur together. In this study, we present a rare case in which the patient has polycystic kidney disease and IAA together and discuss whether IAA is a complication of ADPKD.
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