[Answered]: A 38-year-old male with a PMHx of hypertension…

A 38-year-old male with a PMHx of hypertension and a known history of an inherited disorder involving a missense mutation to the FBN-1 gene presents to the emergency room complaining of an sudden sharp “tearing” chest pain and shortness of breath. On physical examination, the patient is noted to have jugular vein distention and a weak, rapid pulse. His heart rate is noted to be 115 bpm and his blood pressure is 80/40 mmHg. EKG and chest xray are unremarkable. CTA of the chest is suggestive of pericardial effusion. As the patient is awaiting admission for an echocardiogram and most likely surgery, he goes into cardiac arrest and is unable to be resuscitated. Discuss possible findings on autopsy and microscopic examination that would support the patient’s cause of death. Which preventative management strategy might reduce the risk of aortic dissection in a patient with Marfan syndrome?