The Experts below are selected from a list of 12363 Experts worldwide ranked by ideXlab platform
John A. Elefteriades - One of the best experts on this subject based on the ideXlab platform.
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Thoracic Aortic Aneurysm gene dictionary.
Asian cardiovascular & thoracic annals, 2020Co-Authors: Stefanie Rohde, Mohammad A. Zafar, Bulat A. Ziganshin, John A. ElefteriadesAbstract:Thoracic Aortic Aneurysm is typically clinically silent, with a natural history of progressive enlargement until a potentially lethal complication such as rupture or dissection occurs. Underlying g...
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Time to Move to Earlier Intervention for Thoracic Aortic Aneurysm
Structural Heart, 2017Co-Authors: Anton A. Gryaznov, Bulat A. Ziganshin, John A. ElefteriadesAbstract:Aortic disease is the 20th most common cause of death in the U.S. population. One of the most life-threatening complications of Thoracic Aortic Aneurysm (TAA) is acute Aortic dissection and/or rupt...
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Genes Associated with Thoracic Aortic Aneurysm and Dissection
2017Co-Authors: Adam J. Brownstein, Bulat A. Ziganshin, Helena Kuivaniemi, Simon C. Body, Allen E. Bale, John A. ElefteriadesAbstract:Thoracic Aortic Aneurysm (TAA) is a lethal disease, with a natural history of enlarging progressively until dissection or rupture occurs. Since the discovery almost 20 years ago that ascending TAAs are highly familial, our understanding of the genetics of Thoracic Aortic Aneurysm and dissection (TAAD) has increased exponentially. At least 29 genes have been shown to be associated with the development of TAAD, the majority of which encode proteins involved in the extracellular matrix, smooth muscle cell contraction or metabolism, or the transforming growth factor-β signaling pathway. Almost one-quarter of TAAD patients have a mutation in one of these genes. In this review, we provide a summary of TAAD-associated genes, associated clinical features of the vasculature, and implications for surgical treatment of TAAD. With the widespread use of next-generation sequencing and development of novel functional assays, the future of the genetics of TAAD is bright, as both novel TAAD genes and variants within the genes will continue to be identified.
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Guilt by association: paradigm for detecting a silent killer (Thoracic Aortic Aneurysm)
Open heart, 2015Co-Authors: John A. Elefteriades, Adam X. Sang, Gregory A. Kuzmik, Matthew A. HornickAbstract:Recent studies have confirmed a close association between various medical conditions (intracranial Aneurysm, abdominal Aortic Aneurysm, temporal arteritis, autoimmune disorder, renal cysts), certain Aortic anatomic variants (bovine Aortic arch, direct origin of left vertebral artery from Aortic arch, bicuspid Aortic valve), and family history of Aneurysm disease with Thoracic Aortic Aneurysm and dissection. This paper reviews these associations. We propose to capitalise on these associations as powerful and expanding opportunities to diagnose the virulent but silent disease of Thoracic Aortic Aneurysm. This can be accomplished by recognition of this ‘guilt by association’ with the other conditions. Thus, patients with associated diseases and anatomic variants should be investigated for silent Aortic Aneurysms. Such a paradigm holds substantial potential for reducing death from the silent killer represented by Thoracic Aortic Aneurysm disease.
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Practical Genetics of Thoracic Aortic Aneurysm
Progress in cardiovascular diseases, 2013Co-Authors: John A. Elefteriades, Pawel PomianowskiAbstract:This chapter will provide a practical look at the rapidly evolving field regarding the genetics of Thoracic Aortic Aneurysm. It will start with a look at the history of the genetics of Thoracic Aortic Aneurysm and will then move on to elucidating the discovery of familial patterns of Thoracic Aortic Aneurysm. We will next review the Mendelian genetics of transmission of Thoracic Aortic Aneurysm. We will move on to the molecular genetics at the DNA level and finish with a discussion of the molecular genetics at the RNA level, including a promising investigational “RNA Signature” test that we have been developing at Yale.
Robert J Lefkowitz - One of the best experts on this subject based on the ideXlab platform.
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the role of β arrestin2 dependent signaling in Thoracic Aortic Aneurysm formation in a murine model of marfan syndrome
American Journal of Physiology-heart and Circulatory Physiology, 2015Co-Authors: James W Wisler, Emily M Harris, Michael Raisch, Lan Mao, Jihee Kim, Howard A Rockman, Robert J LefkowitzAbstract:This manuscript demonstrates that β-arrestin2 mediates Thoracic Aortic Aneurysm formation in a murine model of Marfan syndrome (MFS) by regulating proAneurysmal signaling. This work identifies a no...
Alan C. Braverman - One of the best experts on this subject based on the ideXlab platform.
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heritable Thoracic Aortic Aneurysm disease recognizing phenotypes exploring genotypes
Journal of the American College of Cardiology, 2015Co-Authors: Alan C. BravermanAbstract:The discovery of a Thoracic Aortic Aneurysm (TAA) or Aortic dissection requires evaluation of relatives for similar disease because 20% will have an affected relative. Heritable TAA diseases are due to mutations in a number of genes that affect the aorta and its branches with differing severity.
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Medical management of Thoracic Aortic Aneurysm disease
The Journal of Thoracic and Cardiovascular Surgery, 2013Co-Authors: Alan C. BravermanAbstract:The patient with Thoracic Aortic Aneurysm disease requires careful evaluation and management over his or her lifetime. This includes assessment for the presence of an underlying genetic disorder, such as Marfan syndrome, bicuspid Aortic valve disease, or a familial Aortic Aneurysm syndrome. Screening family members is necessary, inasmuch as up to 20% of first-degree relatives of the patient with a Thoracic Aortic Aneurysm will also have Aneurysm disease. Medical therapy is often prescribed, and beta-blocker therapy to reduce the stress on the Aortic wall is usually recommended. However, very few clinical trials of pharmacologic therapy in humans with Thoracic Aortic Aneurysm disease have been conducted. Mouse models have led to important discoveries and insight into the pathogenesis of Aneurysm syndromes, and there is hope these may lead to effective therapy in people. Several studies are ongoing that examine the role of angiotensin receptor blockers in Marfan syndrome. Lifestyle modification is also important for patients with Thoracic Aortic Aneurysm, including restrictions on physical activity, weight lifting, and recommendations about the management of pregnancy. Long-term surveillance of the aorta, even after successful surgery, is necessary for timing of prophylactic surgery and to evaluate for late complications.
Hazim J. Safi - One of the best experts on this subject based on the ideXlab platform.
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Successful Descending Thoracic Aortic Aneurysm Repair during a Twin Pregnancy: Case Report and Literature Review
Annals of vascular surgery, 2007Co-Authors: Martin A. Villa, Anthony L. Estrera, Hazim J. SafiAbstract:A 28-year-old woman in the first trimester of a twin pregnancy presented with a symptomatic descending Thoracic Aortic Aneurysm. We report our experience in managing a descending Thoracic Aortic Aneurysm in this patient.
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thoracoabdominal and descending Thoracic Aortic Aneurysm surgery in patients aged 79 years or older
Journal of Vascular Surgery, 2002Co-Authors: Thi Thanh Tam Huynh, Anthony L. Estrera, Charles C Miller, Eyal E Porat, Hazim J. SafiAbstract:Abstract Objective: Advanced age has been reported as a predictor of increased morbidity and mortality in patients who undergo major cardiovascular reconstructive surgery. In this study, we evaluated the outcome of patients aged 79 years or older after thoracoabdominal and descending Thoracic Aortic Aneurysm repair. Methods: From February 1991 to May 2001, 854 patients underwent operation for thoracoabdominal or descending Thoracic Aortic Aneurysm. Fifty-six patients were between the ages of 79 and 88 years at the time of surgery, and these patients were included in this study. Risk factors were analyzed for their impact on mortality and morbidity in these elderly patients with univariate analysis. Results: In patients at low risk, the 30-day mortality rate was 7/42 (17%), compared with 7/14 (50%) in patients at high risk with emergency presentation, congestive heart failure, or diabetes ( P Conclusion: Thoracoabdominal and descending Thoracic Aortic Aneurysm repair in elderly patients can be undertaken with acceptable mortality and morbidity, provided that patients are selected appropriately. (J Vasc Surg 2002;36:469-75.)
Kim A Eagle - One of the best experts on this subject based on the ideXlab platform.
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cardiopulmonary exercise testing following open repair for a proximal Thoracic Aortic Aneurysm or dissection
Journal of Cardiopulmonary Rehabilitation and Prevention, 2020Co-Authors: Whitney E Hornsby, Elizabeth L Norton, Samantha Fink, Sara Saberi, Cheri L Mcgowan, Robert D Brook, Lee Jones, Cristen J Willer, Himanshu J Patel, Kim A EagleAbstract:Purpose: There are limited data on cardiopulmonary exercise testing (CPX) and cardiorespiratory fitness (CRF), following open repair for a proximal Thoracic Aortic Aneurysm or dissection. The aim was to evaluate serious adverse events, abnormal CPX event rate, CRF (peak oxygen uptake, Vo), and blood pressure. Methods: Patients were retrospectively identified from cardiac rehabilitation participation or prospectively enrolled in a research study and grouped by phenotype: (1) bicuspid Aortic valve/Thoracic Aortic Aneurysm, (2) tricuspid Aortic valve/Thoracic Aortic Aneurysm, and (3) acute type A Aortic dissection. Results: Patients (n = 128) completed a CPX a median of 2.9 mo (interquartile range: 1.8, 3.5) following repair. No serious adverse events were reported, although 3 abnormal exercise tests (2% event rate) were observed. Eighty-one percent of CPX studies were considered peak effort (defined as respiratory exchange ratio of ≥1.05). Median measured Vo was <36% predicted normative values (19.2 mL·kgmin vs 29.3 mL·kg·min, P <.0001); the most marked impairment in Vo was observed in the acute type A Aortic dissection group (<40% normative values), which was significantly different from other groups (P <.05). Peak exercise systolic and diastolic blood pressures were 160 mm Hg (144, 172) and 70 mm Hg (62, 80), with no differences noted between groups. Conclusions: We observed no serious adverse events with an abnormal CPX event rate of only 2% 3 mo following repair for a proximal Thoracic Aortic Aneurysm or dissection. Vo was reduced among all patient groups, especially the acute type A Aortic dissection group, which may be clinically significant, given the well-established prognostic importance of reduced cardiorespiratory fitness.
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diagnosis and management issues in Thoracic Aortic Aneurysm
American Heart Journal, 2011Co-Authors: Anna M Booher, Kim A EagleAbstract:Thoracic Aortic enlargement is an increasingly recognized condition that is often diagnosed on imaging studies performed for unrelated indications. The risk of unrecognized and untreated Aortic enlargement and Aneurysm includes Aortic rupture and dissection which carry a high burden of morbidity and mortality. The etiologies underlying Thoracic Aortic enlargement are diverse and can range from degenerative or hypertension associated Aortic enlargement to more rare genetic disorders. Therefore, the evaluation and management of these patients can be complex and requires knowledge of the pathophysiology associated with Thoracic Aortic dilation and Aneurysm. Additionally, there have been important advances in the treatment available to patients with Thoracic Aortic disease, including an increased role of endovascular therapy. Given the risk of mortality, increased clinical recognition and advances in therapeutics, the American College of Cardiology, American Heart Association and related professional societies have recently published guidelines on the management of Thoracic Aortic disease. This review focuses on the pathophysiology and various etiologies that lead to Thoracic Aortic Aneurysm along with the diagnostic modalities and management of asymptomatic patients with Thoracic Aortic disease.
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acute Aortic syndromes and Thoracic Aortic Aneurysm
Mayo Clinic Proceedings, 2009Co-Authors: Vijay S Ramanath, Thoralf M Sundt, Kim A EagleAbstract:Acute and chronic Aortic diseases have been diagnosed and studied by physicians for centuries. Both the diagnosis and treatment of Aortic diseases have been steadily improving over time, largely because of increased physician awareness and improvements in diagnostic modalities. This comprehensive review discusses the pathophysiology and risk factors, classification schemes, epidemiology, clinical presentations, diagnostic modalities, management options, and outcomes of various Aortic conditions, including acute Aortic dissection (and its variants intramural hematoma and penetrating Aortic ulcers) and Thoracic Aortic Aneurysms. Literature searches of the PubMed database were conducted using the following keywords: Aortic dissection, intramural hematoma, Aortic ulcer, and Thoracic Aortic Aneurysm. Retrospective and prospective studies performed within the past 20 years were included in the review; however, most data are from the past 15 years.
