Purpose: To explore the safety and feasibility of stent-graft placement in the dissected descending thoracic aorta of patients with Marfan syndrome.
Methods: Six consecutive patients (4 men; mean age 33+/-15 years, range 24-61) with Marfan syndrome were offered endovascular repair for dissection after previous aortic root repair in 5 and solitary type B dissection in 1.
Results: Transluminal placement of customized Talent stent-grafts was technically successful in all patients, with no 30-day or 1-year intervention-related mortality. Complete abolition of the dissection and reconstruction of the entire dissected aorta was documented in 2 patients. Over a mean 51+/-22-month follow-up (range 12-74), elective conversion to surgical repair was necessary in 2 patients at 22 and 43 months after stent-graft implantation. In a third patient, conversion to surgery is being considered at 74 months after stent-grafting. One patient died suddenly 12 months after endovascular repair.
Conclusions: Nonsurgical reconstruction of postsurgical distal aortic dissection in patients with Marfan syndrome is feasible and technically successful. Stent-graft placement may either avoid or bridge to repeat surgery of distal aortic dissections after previous aortic root repair. Technical expertise and close postinterventional surveillance appear mandatory and may limit the procedure to centers of competence for aortic diseases.
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http://dx.doi.org/10.1583/04-1415MR.1 | DOI Listing |
Diagnostics (Basel)
January 2025
Department of Human Science and Promotion of Quality of Life, San Raffaele Open University, 00163 Rome, Italy.
Marfan syndrome (MFS) is a genetic disorder affecting connective tissue, often leading to cardiovascular complications such as aortic aneurysms and mitral valve prolapse. Cardiovascular multimodality imaging plays a crucial role in the diagnosis, monitoring, and management of MFS patients. This review explores the advancements in echocardiography, cardiovascular magnetic resonance (CMR), cardiac computed tomography (CCT), and nuclear medicine techniques in MFS.
View Article and Find Full Text PDFRadiol Case Rep
March 2025
Radiology Department, University Hospital Center of Souss Massa, Faculty of Medicine and Pharmacy, Ibn Zohr Agadir University, Agadir, Morocco.
Fibromuscular Dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disorder predominantly affecting women aged 18 to 65 years. This case report highlights a 74-year-old female diagnosed with FMD incidentally during evaluation for deep vein thrombosis (DVT). Imaging revealed significant vascular anomalies, including a giant intracranial carotid aneurysm and a hypoplastic iliac vein with extensive collateral formation.
View Article and Find Full Text PDFFront Genet
January 2025
Sichuan Provincial Key Laboratory for Genetic Disease, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, China.
Marfan syndrome (MFS) is an inherited disorder that affects the connective tissues and mainly presents in the bones, eyes, and cardiovascular system, etc. Aortic pathology is the leading cause of death in patients with Marfan syndrome. The fibrillin-1 gene () is a major gene involved in the pathogenesis of MFS.
View Article and Find Full Text PDFJ Thorac Dis
December 2024
Department of Cardiovascular Surgery, Beijing Anzhen Hospital, Capital Medical University, and Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing, China.
Background: Acute type A aortic dissection (ATAAD) requires emergency surgery, but the choice of primary surgery remains controversial. It is believed that simple ascending aorta replacement may lead to higher postoperative survival rate, while the Sun procedure [frozen elephant trunk (FET) + total arch replacement (TAR)] performed in the first stage may obtain better long-term results. The study aimed to compare the outcome of ATAAD patients who underwent the Sun procedure with those without TAR + FET.
View Article and Find Full Text PDFFront Cell Dev Biol
January 2025
Medical Cell Biology Research Group, Department of Human Structure and Repair, Faculty of Medicine and Health Sciences, Ghent University, Ghent, Belgium.
The introduction of pluripotent stem cells into the field of disease modelling resulted in numerous opportunities to study and uncover disease mechanisms in a petri dish. This promising avenue has also been applied to model Marfan syndrome, a disease affecting multiple organ systems, including the skeletal and cardiovascular system. Marfan syndrome is caused by pathogenic variants in , the gene encoding for the extracellular matrix protein fibrillin-1 which ensembles into microfibrils.
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