Recent reports support the role of a valve-sparing procedure in ascending aortic dissection in patients with Marfans syndrome. A 49-year-old woman with Marfans syndrome and prior aortic aneurysm repaired with a composite graft presented with sudden-onset chest pain. Following an initial negative computed tomographic (CT) scan, a long dissection involving the descending thoracic and abdominal aorta was discovered on a repeat CT scan a few hours later. Symptoms improved gradually with optimal medical management and the patient was discharged home on anticoagulant therapy. Although no direct cause-and-effect relationship can be established, chronic anticoagulant therapy may accelerate the progression of recurrent dissection in these patients. A valve-sparing procedure should be considered in eligible patients with Marfans syndrome who need operative correction to avoid possible future untoward effects of long-term anticoagulant therapy.
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http://dx.doi.org/10.1097/01.CRD.0000078443.08887.28 | DOI Listing |
Cureus
November 2024
Anesthesia and Intensive Care, Melegnano Hospital - ASST Melegnano e Martesana, Milan, ITA.
We describe the case of a 72-year-old male suffering from Marfan syndrome, who, because of cardiac abnormalities correlated to the syndrome, received an orthotopic heart transplant four years ago. In 2024, he was diagnosed with right colon cancer. The decision to operate was difficult because of the elevated perioperative risk.
View Article and Find Full Text PDFCureus
November 2024
Cardiovascular Medicine, Hayatabad Medical Complex, Peshawar, PAK.
Introduction and objectives The Bentall procedure is a surgical technique designed to address aortic root abnormalities, including issues with the aortic valve, aortic root, and ascending aortic disease. This study aimed to assess the short-term outcomes of 39 patients who underwent the Bentall and concomitant procedures: aortic root enlargement, personalized external aortic root support (PEARS), and Mini-Bentall procedures at a single center. Methodology We conducted a retrospective study involving 39 patients who underwent surgery for aortic root pathologies such as dissection, Marfan syndrome (MFS), bicuspid aortic valve, degenerative disease, and atherosclerosis at our hospital between January 2019 and September 2024.
View Article and Find Full Text PDFPLoS One
December 2024
McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States of America.
Background: COVID-19 infection and vaccination have been reported to confer an elevated risk for cardiovascular events (CVE). We sought to determine whether individuals with an underlying vascular connective tissue disorder including Marfan syndrome (MFS), Loeys-Dietz syndrome (LDS), or vascular Ehlers Danlos syndrome (vEDS) are at increased risk for cardiac events after COVID-19 infection or vaccination.
Methods: 325 respondents self-reported data through a cross-sectional, web-based survey available from 22 November 2021, through 15 March 2022 regarding COVID-19 illness and vaccinations, the occurrence of any CVE, and adverse events following vaccination.
JAMA Ophthalmol
December 2024
Harkness Eye Institute, Columbia University Medical Center, New York, New York.
Arterioscler Thromb Vasc Biol
December 2024
Department of Surgery (Cardiac), Yale School of Medicine, New Haven, CT. (P.R., B.J., A.H., G.L., W.L., R.A., G.T.).
Background: Smooth muscle cells (SMCs) of cardiac and neural crest origin contribute to the developing proximal aorta and are linked to disease propensity in adults.
Methods: We analyzed single-cell transcriptomes of aortic SMCs from adult mice to determine basal states and changes after disrupting TGFβ (transforming growth factor-β) signaling necessary for aortic homeostasis.
Results: A minority of Myh11 lineage-marked SMCs differentially expressed genes suggestive of embryological origin.
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