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<p>Marfan syndrome (MFS) is a hereditary connective tissue disorder, primarily affecting the cardiovascular, skeletal, and ocular systems. Cardiovascular complications, particularly aortic aneurysm and dissection, are the leading causes of morbidity and mortality in these patients. We present the case of a 45-year-old woman with MFS who underwent a Bentall-De Bono procedure for type B aortic dissection. She later developed Staphylococcus aureus prosthetic valve endocarditis, complicated by periprosthetic infection and embolic events. Despite long-term suppressive antibiotic therapy, she discontinued treatment due to gastrointestinal intolerance. Follow-up imaging, including echocardiography, PET/CT, and CT angiography, revealed an unchanged aneurysm in the descending thoracic aorta with a dissection flap but no active infection. Given the absence of new complications, conservative management was chosen. This case highlights the importance of multimodal imaging and individualized treatment strategies in MFS patients with complex cardiovascular history.</p>

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Diffuse vasculopathy in a patient with Marfan syndrome

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年6月11日

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  • Teresa Perillo,
  • Claudia Giorgio,
  • Carmine Frasca,
  • Lorenzo Ugga,
  • Antonio Pinto

摘要

Marfan syndrome (MFS) is a hereditary connective tissue disorder, primarily affecting the cardiovascular, skeletal, and ocular systems. Cardiovascular complications, particularly aortic aneurysm and dissection, are the leading causes of morbidity and mortality in these patients. We present the case of a 45-year-old woman with MFS who underwent a Bentall-De Bono procedure for type B aortic dissection. She later developed Staphylococcus aureus prosthetic valve endocarditis, complicated by periprosthetic infection and embolic events. Despite long-term suppressive antibiotic therapy, she discontinued treatment due to gastrointestinal intolerance. Follow-up imaging, including echocardiography, PET/CT, and CT angiography, revealed an unchanged aneurysm in the descending thoracic aorta with a dissection flap but no active infection. Given the absence of new complications, conservative management was chosen. This case highlights the importance of multimodal imaging and individualized treatment strategies in MFS patients with complex cardiovascular history.