Background: Beta-blockers are the standard treatment in Marfan syndrome (MFS). Recent clinical trials with limited follow-up yielded conflicting results on losartan's effectiveness in MFS.
Objectives: The present study aimed to evaluate the benefit of losartan compared with atenolol for the prevention of aortic dilation and complications in Marfan patients over a longer observation period (>5 years).
Methods: A total of 128 patients included in the previous LOAT (LOsartan vs ATenolol) clinical trial (64 in the atenolol and 64 in the losartan group) were followed up for an open-label extension of the study, with the initial treatment maintained.
Results: Mean clinical follow-up was 6.7 ± 1.5 years. A total of 9 events (14.1%) occurred in the losartan group and 12 (18.8%) in the atenolol group. Survival analysis showed no differences in the combined endpoint of need for aortic surgery, aortic dissection, or death (p = 0.462). Aortic root diameter increased with no differences between groups: 0.4 mm/year (95% confidence interval: 0.2 to 0.5) in the losartan and 0.4 mm/year (95% confidence interval: 0.3 to 0.6) in the atenolol group. In the subgroup analyses, no significant differences were observed considering age, baseline aortic root diameter, or type of dominant negative versus haploinsufficient FBN1 mutation.
Conclusions: Long-term outcome of Marfan syndrome patients randomly assigned to losartan or atenolol showed no differences in aortic dilation rate or presence of clinical events between treatment groups. Therefore, losartan might be a useful, low-risk alternative to beta-blockers in the long-term management of these patients.
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http://dx.doi.org/10.1016/j.jacc.2018.07.052 | DOI Listing |
Turk Kardiyol Dern Ars
January 2025
Department of Cardiology, Gülhane Faculty of Medicine, University of Health Sciences, Ankara, Türkiye.
Severe mitral regurgitation (MR) following surgical repair of the mitral valve poses a significant clinical challenge. Patients who have undergone surgery are typically at high risk for a second operation. This report details the case of a 54-year-old male who underwent aortic valve replacement and mitral valve repair using a 34-ring, 14 years prior.
View Article and Find Full Text PDFInt J Mol Sci
December 2024
Department of Vascular Surgery, RWTH Aachen University Hospital, 52074 Aachen, Germany.
Thoracoabdominal aortic aneurysms (TAAAs) are rare but serious conditions characterized by dilation of the aorta characterized by remodeling of the vessel wall, with changes in the elastin and collagen content. Individuals with Marfan syndrome have a genetic predisposition for elastic fiber fragmentation and elastin degradation and are prone to early aneurysm formation and progression. Our objective was to analyze the medial collagen characteristics through histological, polarized light microscopy, and electron microscopy methods across the thoracic and abdominal aorta in twenty-five patients undergoing open surgical repair, including nine with Marfan syndrome.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
September 2024
Department of Pediatric Cardiovascular Surgery, Kanazawa Medical University, Ishikawaken, Japan.
Background: The study focuses on vascular compression of the main bronchus in the aortopulmonary space, examining potential contributors within the same axial plane. Its goal is to uncover mechanisms of bronchial compression in patients with intracardiac anomalies and review surgical outcomes, aiming to enhance future results.
Methods: The morphology and topology of structures within the axial plane of the aortopulmonary space were objectively analyzed, including the sternum, ascending aorta, heart, pulmonary artery, descending aorta, and other relevant elements.
Cardiovasc Diagn Ther
December 2024
Department of Cardiology, St. Luke's International Hospital, Tokyo, Japan.
Tetralogy of Fallot (TOF) is a condition that often leads to long-term enlargement of the aortic root in after surgery. The aortic dilation is believed to be caused by histological abnormalities of the aortic media and the hemodynamic characteristics of increased aortic flow, compared to pulmonary flow. Severe cyanosis, severe right ventricular outflow tract (RVOT) obstruction, older age at repair, a larger aortic size at the time of repair, and a history of an aortopulmonary shunt parameters related to long-standing volume overload of the aortic root were the reported risk factors.
View Article and Find Full Text PDFCardiol Young
January 2025
Department of Pediatrics, Oregon Health and Science University, Portland, OR, USA.
Echocardiographic Z-score models play a crucial role in defining cardiac pathology in paediatric patients. There are multiple models that practitioners utilize in the United States without guiding principles to standardize their use. Discrepant interpretations can occur depending on the model chosen, even if standardized Z-score cutoffs are applied.
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