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http://dx.doi.org/10.1016/j.jacc.2016.11.068 | DOI Listing |
Obstet Med
July 2025
Faculty of Medicine, The University of Queensland, Herston, Queensland, Australia.
Acute aortic dissection related to pregnancy is rare, however represented the third most common cause of pregnancy-related cardiovascular death in the 2017 UK-MBBRACE report. The majority of women with pregnancy-related aortic dissection have an underlying inherited syndromic aortopathy, though this is often not recognised until an event. Data regarding the immediate and long-term effects of pregnancy on aortic outcome are not uniform due to publication and ascertainment biases, small sample sizes, inclusion of women whose dissection was the first presentation of the underlying disease, and incompleteness of collected data.
View Article and Find Full Text PDFJACC Adv
August 2025
Department of Internal Medicine, McGovern Medical School at UTHealth Houston, Houston, Texas, USA. Electronic address:
Thoracic aortic aneurysms predisposing to aortic dissections are rare but potentially deadly conditions that can be inherited in families. Understanding the natural history and genetic causes of thoracic aortic disease (TAD) requires international collaboration. The aim of this project is to provide core data elements that can be assessed directly and summarized efficiently in research records while developing standards to harmonize data across registries and clinical trials.
View Article and Find Full Text PDFInt J Cardiol Heart Vasc
June 2025
Department of Molecular & Clinical Medicine, Ninewells Hospital & Medical School, University of Dundee, Dundee DD1 9SY, UK.
Bicuspid Aortic Valve (BAV) is the most prevalent congenital heart defect with an autosomal dominant inheritance. With up to 2% of the general population affected by the condition, mortality remains high likely due to the development of aortopathy which pre-disposes to the development of aortic dissection. Current guidelines focus on surgical management, once a threshold of aorta diameter is surpassed, via routine image surveillance.
View Article and Find Full Text PDFAnn Med Surg (Lond)
February 2025
Plateau State Specialist Hospital Jos Nigeria, Plateau State, Nigeria.
Inherited aortopathies, including Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome, are genetic disorders characterized by structural abnormalities of the aorta that predispose individuals to life-threatening complications like aneurysms and dissections. These conditions result from mutations in genes essential for maintaining aortic wall integrity, such as FBN1, TGFBR1, and COL3A1, affecting extracellular matrix components and the transforming growth factor-beta (TGF-β) pathway. Marfan syndrome has a prevalence of approximately 1 in 5000, while Loeys-Dietz syndrome and vascular Ehlers-Danlos syndrome are rarer, with estimated prevalences of 1 in 100 000 and 1 in 20 000, respectively.
View Article and Find Full Text PDFDiagnostics (Basel)
March 2025
Department of Cardiovascular Surgery, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China.
Congenital bicuspid aortic valve (BAV) signifies the most frequent category of congenital cardiovascular anomaly globally, occurring in approximately 0.5-2% of the general population worldwide. BAV is a major cause of thoracic aortopathy, encompassing aortic stenosis, aortic root dilation with regurgitation, aortic dissection, and aortic aneurysms, consequently leading to substantial late-onset morbidity and mortality.
View Article and Find Full Text PDF