Pathophysiology
Clinical meaning
Marfan syndrome is an autosomal dominant connective tissue disorder caused by mutations in the FBN1 gene encoding fibrillin-1. Defective fibrillin weakens elastic fibers in the aorta, eyes, and skeleton, leading to aortic root dilation and risk of dissection or rupture. Connect Marfan Syndrome to bedside cues you will reassess first: vitals trends, work of breathing, perfusion, mentation, and pain or ischemic equivalents when relevant. Boards reward recognizing when subtle instability outweighs reassurance, then selecting nursing actions that protect airway, circulation, and neurologic status before routine tasks.