Pathophysiology
Clinical meaning
Defective fibrillin-1 impairs elastic fiber assembly in the aortic media, leading to cystic medial necrosis. The weakened aortic wall progressively dilates at the sinuses of Valsalva, creating an aneurysm prone to dissection. TGF-beta dysregulation from abnormal fibrillin accelerates smooth muscle apoptosis and extracellular matrix degradation. Connect Marfan Cardiac 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.