Review Question - QID 106401

This patient with Marfan syndrome and a family history of possible sudden death from aortic disease most likely has a thoracic aortic dissection, which should be evaluated with CT angiography of the aorta.

Aortic dissection occurs when a tear in the inner wall of the aorta causes blood to flow between the layers of the aorta, forcing the layers apart. Most patients present with severe chest or abdominal pain that is "tearing" in character, and often with other symptoms that result from decreased blood supply to other organs. Aortic dissection is a medical emergency and can quickly lead to death, even with optimal treatment, due to decreased blood supply to other organs, cardiac failure, and sometimes rupture of the aorta. Risk factors for aortic dissection include hypertension, known thoracic aortic aneurysm, and connective tissue disorders such as Marfan syndrome and Ehlers–Danlos syndrome. The diagnosis is made with medical imaging (computed tomography, magnetic resonance imaging or transesophageal echocardiography).

McConaghy and Oza discuss the outpatient diagnosis of acute chest pain. Pneumonia, heart failure, pulmonary embolism, acute pericarditis, and acute thoracic aortic dissection (acute chest or back pain with a pulse differential in the upper extremities) are part of the differential diagnosis.

Crawford reviews the diagnosis and management of aortic dissection. Diagnosis is confirmed by computed tomography, aortography, or echocardiography. Successful outcomes depend on quickly recognizing the symptoms of dissection, prompt diagnosis, and early administration of appropriate treatment. Medical management includes controlling blood pressure and decreasing left ventricular contractility.

Figure A is a photograph of pectus excavatum, a sunk-in appearing chest that occurs more frequently in individuals with Marfan syndrome. Figure B is a photograph of arachnodactyly, long fingers seen more commonly in patients with Marfan syndrome. Illustration A is a CT scan with contrast showing an aortic dissection. Note the two lumina separated by an intimal flap.

Incorrect Answers:
Answer 1: While a 12-lead electrocardiogram is essential in the work-up of acute chest pain, imaging of the thoracic aorta should be the highest priority in a patient with a high likelihood of aortic dissection.
Answer 2: While transesophageal echocardiography is a useful diagnostic test for aortic dissection, transthoracic echocardiography is less useful.
Answer 4: Coronary catheterization would be appropriate if acute coronary syndrome were the most likely diagnosis.
Answer 5: IV contrast is necessary to visualize the true and false lumina of the aorta in the case of a dissection.