Can Frozen Elephant Trunk Cure Type I Dissection Confined to Thoracic Aorta in Marfan Syndrome? (Commentary)

Publication date: Available online 28 September 2019Source: The Annals of Thoracic SurgeryAuthor(s): Jehangir J. Appoo
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research

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This study examines the impact of presentation and demographics on late survival in patients with MFS.
Source: Journal of Vascular Surgery - Category: Surgery Authors: Source Type: research
Source: - Category: General Medicine Source Type: news
This article will provide an overview of the literature on potential biomarkers studied so far in MFS, as well as potential future directions.
Source: Cardiology in Review - Category: Cardiology Tags: Review Articles Source Type: research
Acute aortic dissection can represent one of the most complex diseases treated by vascular surgeons. Presented is a 39-year-old female with Marfan Syndrome and previous aortic interventions with acute type-A aortic dissection complicated by visceral and cerebral malperfusion. The case was further complicated by twin pregnancy, active anticoagulation for mechanical aortic valve, ectopia lentis syndrome of the right eye and TIA involving the left eye, and rupture of the remaining native ascending aorta requiring cardiopulmonary bypass and hypothermic circulatory arrest with a 26-week twin pregnancy.
Source: Annals of Vascular Surgery - Category: Surgery Authors: Source Type: research
This study aimed to introduce a simple screening method for identifying aortic root dilation in children. The study population consisted of 190 children who were diagnosed with Marfan syndrome or Marfan-like disorders. The aortic root ratio (ARr) was formulated. The value of the ARr was compared in each patient with the results inz-scores, which were obtained using on-line calculators based on the most widespread nomograms. The optimal cut-off value of the ARr was ≥ 18.7. At this cut-off point, the sensitivity of the ARr ranged from 88.3% to 100% and the specificity ranged from 94% to 97.8%. All of the patients i...
Source: Pediatric Cardiology - Category: Cardiology Source Type: research
This report extends the phenotypic spectrum of variants identified in theSKI gene. We describe a new mutational hotspot associated with a marfanoid syndrome with no intellectual disability. Cardiovascular involvement was confirmed in a significant number of cases, highlighting the importance of accurately diagnosing SGS and ensuring appropriate medical treatment and follow-up.
Source: Human Genetics - Category: Genetics & Stem Cells Source Type: research
Publication date: February 2020Source: The Annals of Thoracic Surgery, Volume 109, Issue 2Author(s): Sherene Shalhub, Mary J. Roman, Kim A. Eagle, Scott A. LeMaire, Qianzi Zhang, Artur Evangelista, Dianna M. Milewicz, Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) ConsortiumBackgroundTo investigate clinical course of patients with type B aortic dissection (TBAD) occurring at a young age with confirmed or suspected heritable thoracic aortic disease.MethodsIndividuals with TBAD occurring at an age
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
ConclusionAlthough compound heterozygosity or homozygosity is rare in MFS, it should be considered when there is an unusually severe phenotype in a subset of family members.
Source: Molecular Genetics & Genomic Medicine - Category: Genetics & Stem Cells Authors: Tags: CLINICAL REPORT Source Type: research
CONCLUSIONS: All together, our results suggest sterile inflammation as a novel paradigm to disease progression, and we identify, for the first time, monocytes as a viable candidate for targeted therapy in MVD. PMID: 31928435 [PubMed - in process]
Source: Circulation - Category: Cardiology Authors: Tags: Circulation Source Type: research
Jeremy Norton, from Wigston, Leicestershire, has Marfan syndrome, where the connective tissue throughout the body is too fragile. As a result, the walls of the aorta can become weakened.
Source: the Mail online | Health - Category: Consumer Health News Source Type: news
More News: Cardiovascular & Thoracic Surgery | Marfan Syndrome