Objectives: To evaluate the mid-term outcomes, and the aortic remodeling in Marfan syndrome (MFS) patients with type B dissection that were treated with endovascular repair.
Background: MFS is a relative contraindication to thoracic endovascular aortic repair (TEVAR). Mid-term aortic outcomes data in MFS after TEVAR are limited, and the occurrence of late events remains unclear.
Methods: Of 89 patients that underwent TEVAR between September 2002 and February 2011, 10 patients with mid-term follow-up fulfilled the Ghent criteria for MFS and complicated type B dissection. High risk for open surgery was documented in 90%.
Results: The mean age was 35.1 ± 9.4 years and all patients presented with acute aortic syndrome complicating a chronic type B dissection (DeBakey type IIIb). Five patients underwent a Bentall surgical procedure previous to endovascular repair, and in four patients initial TEVAR was followed by surgery of the ascending aorta. Treatment was limited to endovascular repair in only one patient. In-hospital mortality was 10%. At a mean follow-up of 59.6 ± 38.9 months, the cumulated mortality was of 20% and late mortality 11.1%. The rate of secondary endoleak was 44.4%, and late reintervention of 33.3%. Survival freedom from cardiovascular death at 8 years was 80.0%, and positive remodeling was documented in 37.5% of patients.
Conclusions: Our results suggest that TEVAR is feasible, safe, and associated with a high reintervention rate and reduced rate of positive aortic remodeling in patients with Marfan syndrome. Survival at 8 years was comparable to contemporary series of open repair.
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http://dx.doi.org/10.1002/ccd.24725 | DOI Listing |
J Clin Med
January 2025
Department of Medical Rehabilitation and Clinical Physiotherapy, Pomeranian Medical University, 70-204 Szczecin, Poland.
The objective of this study was to assess the course of rehabilitation of patients hospitalized in the cardiac rehabilitation unit after surgery for acute Stanford type A aortic dissection, extending beyond the ascending aorta, and comparing these findings with those for patients who, after the same type of surgery, had no remaining dissection. The aim was to develop an optimal cardiac rehabilitation model for this patient population, given the lack of clear guidelines. Additionally, the study aimed to evaluate their one-year survival.
View Article and Find Full Text PDFDiagnostics (Basel)
December 2024
Department of Medical Imaging and Intervention, Chang Gung Memorial Hospital, Taoyuan City 333423, Taiwan.
Background/objectives: To develop and validate a model system using deep learning algorithms for the automatic detection of type A aortic dissection (AD), and differentiate it from normal and type B AD patients.
Methods: In this retrospective study, a deep learning model is developed, based on aortic computed tomography angiography (CTA) scans of 498 patients using training, validation and test sets of 398, 50 and 50 patients, respectively. An independent test set of 316 patients is used to validate and evaluate its performance.
Can J Cardiol
January 2025
Cardiovascular department, Cardiology Research Institute, Tomsk National Research Medical Centre, Russian Academy of Sciences, Tomsk, Russian Federation.
Background: The aim of the study was to analyze the mid-term outcomes of the frozen elephant trunk (FET) procedure for chronic aortic dissection (СAD).
Methods: From March 2012 to December 2022, 123 FET procedures were performed in patients with acute and chronic aortic dissection as well as aortic aneurysm. Fifty-five patients with chronic aortic dissection (CAD) were eligible for study.
Sci Immunol
January 2025
Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
Human recombination-activating gene (RAG) deficiency can manifest with distinct clinical and immunological phenotypes. By applying a multiomics approach to a large group of -mutated patients, we aimed at characterizing the immunopathology associated with each phenotype. Although defective T and B cell development is common to all phenotypes, patients with hypomorphic variants can generate T and B cells with signatures of immune dysregulation and produce autoantibodies to a broad range of self-antigens, including type I interferons.
View Article and Find Full Text PDFInt J Hematol
January 2025
Associated Department With Mie Graduate School of Medicine, Mie Prefectural General Medical Center, Yokkaichi, Japan.
This study discusses disseminated intravascular coagulation (DIC) associated with solid cancers and various vascular abnormalities, both of which generally exhibit chronic DIC patterns. Solid cancers are among the most significant underlying diseases that induce DIC. However, the severity, bleeding tendency, and progression of DIC vary considerably depending on the type and stage of the cancer, making generalization difficult.
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