Aortic incompetence in Marfan's syndrome results from distortion or dilatation of the sinuses of Valsalva, annuloaortic ectasia or a combination of these problems. Valve leaflets in these patients are macroscopically normal in spite of aortic insufficiency. Replacement of the ascending aorta, root and aortic valve with a composite graft was, for a long time, the treatment of choice for Marfan patients. Valve-preserving procedures (remodeling or reimplantation) provide the advantages of avoiding the shortcomings of standard surgical techniques, and maintaining the functional integrity of the left ventricular (LV) outflow tract, aortic root and ascending aorta. We developed a modified valve-sparing reimplantation technique for avoiding leaflet damage. This was achieved by leaving a 'cushion' of aortic wall (8--10 mm) that, sewn on the Dacron graft, works as a 'damper' and prevents leaflets injury during the systolic opening of the valve. For final judgment of this operative method long-term results are necessary.
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http://dx.doi.org/10.1016/s0967-2109(00)00142-3 | 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.
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