A 28-year-old Indian male presents to the cardiology clinic with a family history of sudden cardiac death. On examination, the structure marked **A** (arachnodactyly with positive thumb sign) is noted, along with tall stature and arm span exceeding height. Echocardiography reveals aortic root dilatation (Z-score 2.5). Which of the following best explains the pathophysiology underlying this patient's clinical presentation?
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