Aortic Surgery Outcomes of Marfan Syndrome and Ehlers-Danlos Syndrome Patients at Teaching and Nonteaching Hospitals

Despite improvements in prevention and management, aortic aneurysm repair remains a high-risk operation for patients with Marfan syndrome (MFS) and Ehlers-Danlos syndrome (EDS). The goal of this study was to examine differences in characteristics and outcomes of patients with MFS or EDS undergoing aortic aneurysm repair at teaching versus nonteaching hospitals.
Source: Annals of Vascular Surgery - Category: Surgery Authors: Tags: Clinical Research Source Type: research

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Abstract OBJECTIVES: Marfan syndrome and Ehlers-Danlos syndrome represent two connective tissue vascular diseases requiring unique consideration in their vascular surgical care. A comprehensive national review encompassing all hospitalizations for the Marfan Syndrome and Ehlers-Danlos syndrome patient population is lacking. METHODS: The National (Nationwide) Inpatient Sample from 2010 to 2014 was reviewed for all inpatient vascular surgery procedures including those with a diagnosis of Marfan syndrome and Ehlers-Danlos syndrome. National estimates of vascular surgery rates were generated from provided weights...
Source: Vascular - Category: Surgery Authors: Tags: Vascular Source Type: research
BACKGROUND AND PURPOSE: There is a general assumption in the cerebrovascular literature that there is an association between carotid artery tortuosity and connective tissues disease; however, this has not been firmly established. The purpose of this study was to determine the prevalence of carotid artery tortuosity in patients with connective tissue diseases relative to matched controls. MATERIALS AND METHODS: Patients with previous CTA or MRA and a diagnosis of connective tissue diseases were identified and compared with a cohort of age-matched controls. Radiologists blinded to the diagnosis reviewed the images and evalu...
Source: American Journal of Neuroradiology - Category: Radiology Authors: Tags: EXTRACRANIAL VASCULAR Source Type: research
In 2003, a 16-year-old girl underwent a Bentall procedure after development of progressive dyspnea caused by an aneurysm of the ascending aorta and severe aortic regurgitation. Despite aortic aneurysm and retrognathia, no clinical findings of Marfan syndrome or Ehlers-Danlos syndrome were present. After the Bentall procedure, annual follow-up visits, including annual magnetic resonance angiography of the aorta, were performed, revealing, in 2007, an asymptomatic aneurysm of the superior mesenteric artery measuring 14 mm, with a progression to 20 mm in 2016.
Source: Mayo Clinic Proceedings - Category: Internal Medicine Authors: Tags: Medical image Source Type: research
Abstract Recent advances in DNA sequencing technology have identified several causative genes for hereditary thoracic aortic aneurysms and dissections (TAADs), including Marfan syndrome (MFS), Loeys-Dietz syndrome, vascular Ehlers-Danlos syndrome, and familial non-syndromic TAADs. Syndromic TAADs are typically caused by pathogenic variants in the transforming growth factor-β signal and extracellular matrix-related genes (e.g. FBN1, TGFBR1, TGFBR2, SMAD3, TGFB2, and COL3A1). On the other hand, approximately 20% of the non-syndromic hereditary TAADs result from altered components of the contractile apparatus of...
Source: Journal of Cardiology - Category: Cardiology Authors: Tags: J Cardiol Source Type: research
Guillaume Goudot1, Tristan Mirault2,3, Patrick Bruneval2,4, Gilles Soulat5, Mathieu Pernot1 and Emmanuel Messas2,3* 1INSERM U1273, ESPCI Paris, CNRS FRE 2031, Physics for Medicine Paris, PSL Research University, Paris, France 2Centre de Référence des Maladies Vasculaires Rares, Hôpital Européen Georges-Pompidou, Assistance Publique – Hôpitaux de Paris (APHP), Paris, France 3INSERM U970 PARCC, Paris Descartes University – Sorbonne Paris Cité University, Paris, France 4Service d’Anatomie Pathologique, Hôpital Européen Georges-Pompidou, Assistanc...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
Authors: Thijssen CGE, Bons LR, Gökalp AL, van Kimmenade RRJ, Mokhles MM, Pelliccia A, Takkenberg JJM, Roos-Hesselink JW Abstract INTRODUCTION: Current guidelines recommend patients with thoracic aortic disease (TAD) including inherited aortopathies to avoid heavy exercise. However, evidence supporting the negative advice on exercise is scarce. We aimed to provide an up-to-date systematic review of the available evidence on risks and benefits of exercise and sports participation in TAD patients. Areas covered: A systematic search was performed in Medline, Embase and Web of Science: thoracic aortic aneurysm or ...
Source: Expert Review of Cardiovascular Therapy - Category: Cardiology Tags: Expert Rev Cardiovasc Ther Source Type: research
Retrospective review of a National Inpatient Sample from 2000 to 2014.
Source: Journal of Vascular Surgery - Category: Surgery Authors: Tags: Patients with aortic aneurysms and connective tissue disorders do as well at teaching hospitals as at nonteaching hospitals: An excellent summary of a challenging problem Source Type: research
Despite improvements in prevention and management, aortic aneurysm repair remains a high risk operation for patients with Marfan syndrome (MFS) and Ehlers-Danlos syndrome (EDS). The goal of this study was to examine differences in characteristics and outcomes of patients with MFS or EDS undergoing aortic aneurysm repair at teaching versus non-teaching hospitals.
Source: Annals of Vascular Surgery - Category: Surgery Authors: Source Type: research
Aortopathies pose a significant healthcare burden due to excess early mortality, increasing incidence, and underdiagnosis. Understanding the underlying genetic causes, early diagnosis, timely surveillance, prophylactic repair, and family screening are keys to addressing these diseases. Next-generation sequencing continues to expand our understanding of the genetic causes of heritable aortopathies, rapidly clarifying their underlying molecular pathophysiology and suggesting new potential therapeutic targets. This review will summarize the pathogenetic mechanisms and management of heritable genetic aortopathies with attentio...
Source: Clinical Medicine Insights: Cardiology - Category: Cardiology Authors: Source Type: research
We report a case of a 46 year old smoker who had developed multiple aneurysms of the aorta in both the thoracic and abdominal parts and was incidentally diagnosed on work-up of a chronic back pain associated with venous prominence on left side of chest and left arm. PMID: 26591150 [PubMed - indexed for MEDLINE]
Source: Journal of the Association of Physicians of India - Category: Journals (General) Tags: J Assoc Physicians India Source Type: research
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