Bicuspid aortic valve disease is the most common congenital cardiac disorder, being present in 1% to 2% of the general population. Associated aortopathy is a common finding in patients with bicuspid aortic valve disease, with thoracic aortic dilation noted in approximately 40% of patients in referral centers. Several previous consensus statements and guidelines have addressed the management of bicuspid aortic valve-associated aortopathy, but none focused entirely on this disease process. The current guidelines cover all major aspects of bicuspid aortic valve aortopathy, including natural history, phenotypic expression, histology and molecular pathomechanisms, imaging, indications for surgery, surveillance, and follow-up, and recommendations for future research. It is intended to provide clinicians with a current and comprehensive review of bicuspid aortic valve aortopathy and to guide the daily management of these complex patients.
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http://dx.doi.org/10.1016/j.jtcvs.2018.02.115 | DOI Listing |
JACC Cardiovasc Interv
December 2024
Division of Cardiology, University of Ottawa Heart Institute, Ottawa, Ontario, Canada.
JACC Cardiovasc Interv
December 2024
Department of Cardiology, Keio University School of Medicine, Tokyo, Japan.
Background: Evidence regarding the incidence of prosthesis-patient mismatch (PPM) and long-term mortality after transcatheter aortic valve replacement (TAVR) in patients with bicuspid aortic valve stenosis (AS) is scarce.
Objectives: This study sought to assess the incidence and prognostic impact of PPM after TAVR for bicuspid AS compared with that for tricuspid AS.
Methods: In total, 7,393 patients who underwent TAVR were prospectively enrolled in the OCEAN-TAVI (Optimized Catheter Valvular Intervention Transcatheter Aortic Valve Implantation) registry, an ongoing Japanese, multicenter registry.
Am J Med Genet C Semin Med Genet
January 2025
Medical Genetics, Department of Pediatrics, Mass General for Children, Boston, Massachusetts, USA.
Determining karyotype-phenotype correlations for individuals with Turner syndrome ("TS individuals") is a longstanding research endeavor. The limited literature on Turner syndrome (TS) with a ring X chromosome hinders counseling about the neuropsychological and clinical features. To further characterize these phenotypes, we compared 27 TS individuals with 46,X,r(X)/45,X ("ring X") to 50 non-mosaic 45,X, and 27 mosaic 45,X/46,XX ("mosaic 45,X") individuals.
View Article and Find Full Text PDFAnn Cardiol Angeiol (Paris)
January 2025
Service de cardiologie, Centre Hospitalier Universitaire de Tivoli, 34, Avenue Max Buset, 7100 La Louvière, Belgique.
Case Report: We report the case of a 63-year-old patient who underwent aortic valve replacement with a biological valve for a bicuspid aortic stenosis, and LIMA-IVA single-bypass surgery. Two weeks later, he presented with Enterococcus faecillis bacteremia, attributed to left pyelonephritis and successfully treated with Amoxicillin. Two months after his surgery, he had a new bacteremia due to Enterococcus faecalis and we discovered a pseudo-aneurysm of the mitro-aortic trigone.
View Article and Find Full Text PDFAm J Cardiol
January 2025
Department of Cardiovascular Research, Baylor Scott & White Research Institute, Plano, TX.
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