Article ID Journal Published Year Pages File Type
2659803 The Journal for Nurse Practitioners 2014 9 Pages PDF
Abstract

•Marfan syndrome is associated with aortic dilation and dissection.•Main goal of medical therapy is blood pressure control and lifestyle modification.•Prophylactic surgical intervention remains the most definitive measure in preventing aortic dissection or rupture.•Guidelines are available for clinical diagnosis and management of Marfan syndrome.

Marfan syndrome (MFS) is a genetic disorder affecting 1 in 5,000 individuals. The diagnosis is made using a combination of genetic testing and the revised Ghent criteria. MFS is associated with the cardiovascular-related risks of aortic dilation and dissection. Therefore, the main goal of medical therapy is blood pressure control using beta-blockers and lifestyle modification. Prophylactic surgical intervention remains the single, definitive measure in preventing dissection or rupture. Nurse practitioners must be vigilant in caring for this population as these cardiovascular risks can be reduced by early identification and diagnosis and timely intervention.

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