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Prevention of Cardiovascular Complications in the Marfan Syndrome

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Abstract:

Marfan syndrome is an autosomal dominant inherited connective tissue disorder with an estimated prevalence of 2-3 per 10,000. The underlying genetic cause of the Marfan syndrome is a mutation in the fibrillin I gene at chromosome 15. The manifestation of Marfan syndrome primarily involves the cardiovascular, musculoskeletal and ocular systems. Patients with Marfan syndrome are at risk of sudden death at young age due to aortic dilatation and dissection in the entire aorta, which are not always predictable by mere anatomic assessment of the aortic diameter. Recently it has been shown that both aortic stiffness and aortic diameters are independent predictors for aortic complications. Due to improved detection of patients at risk, timely surgical intervention may prevent aortic dissection and death. Currently, it is possible to replace the entire aorta with a Dacron graft. The role of ß-blocking therapy is well established in non-operated patients, but seems doubtful in patients with a substantial part of the aorta replaced by an artificial graft. Prophylactic aortic surgery should be considered in pregnant women with Marfan syndrome and enlarged aortic diameters, because of the increased risk of dissection.

In this review we will describe cardiovascular complications of the Marfan syndrome and will discuss strategies for prevention and management.

Keywords: aortic surgery; marfan syndrome; prevention; review

Document Type: Review Article

DOI: http://dx.doi.org/10.2174/1567270043480850

Affiliations: Department of Cardiology, Room B2-240, Academic Medical Center, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands.

Publication date: March 1, 2004

More about this publication?
  • Vascular Disease Prevention publishes reviews as well as original papers to update all those concerned with this topic at the clinical or scientific level. In addition to clinically relevant topics, we consider reviews and original papers dealing with the more scientific aspects of vascular disease prevention. This includes the evaluation of emerging vascular risk factors, research dealing with the pathogenesis of atherosclerosis and the investigation of new treatment options both at the clinical and scientific level (e.g. epidemiology, patient-based studies, experimental models, in vitro experiments or molecular research). Therefore, another function of Vascular Disease Prevention is to bridge the gap between clinical practice and ongoing laboratory-based research.

    In particular, we welcome critical reviews and comments on recent trials. This is a topic that requires regular updates because of the large number of trials published every year.

    Debates are encouraged in the correspondence section of this journal.
    The editorial structure of Vascular Disease Prevention is set up with the aim of dealing with the submitted material as rapidly as possible. Specialist editors will provide a more expert and rapid assessment unlike a more centralized editorial structure.
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