Review Question - QID 109120

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Marfan Syndrome QID: 109120 (M2.OR.17.4774)

QID 109120 (Type "109120" in App Search)

A 36-year-old male is brought to the emergency department by fire and rescue after being found down in his apartment by his wife. His wife reports that the patient had been complaining of chest pain for the last few hours but refused to go to the hospital. She went to the grocery store for about half an hour and found her husband unresponsive on the ground when she returned home. The patient’s wife reports that his past medical history is significant for a “heart murmur” and that the patient’s father died at age 32 for unknown reasons. In the trauma bay, the patient’s temperature is 98.8°F (37.1°C), blood pressure is 88/41 mmHg, pulse is 116/min, and respirations are 12/min. On physical exam, the patient has a Glascow Coma Score (GCS) of 7. He has a tall stature with long limbs and long, slender fingers. He is also noted to have a pectus deformity. On cardiac exam, the patient has a diastolic decrescendo murmur at the left third intercostal space. He has weak brachial and femoral pulses.

Which of the following is the most likely etiology of this patient’s presentation?

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Coronary atherosclerosis

0/82

Hypertrophic cardiomyopathy

1/82

Spontaneous rupture of the aorta

48%

39/82

Spontaneous rupture of the mitral chordae tendinae

7/82

Spontaneous separation in the arterial wall

39%

32/82

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This patient presents with tall stature, long limbs and fingers, pectus deformity, and a family history of early death, suggesting a diagnosis of Marfan's syndrome. The symptoms of syncope, chest pain, hypotension with wide pulse pressure, and weak peripheral pulses suggest a diagnosis of aortic dissection (separation in the arterial wall).

Marfan's syndrome presents with several cardiac pathologies, including aortic root aneurysm, aortic regurgitation, aortic dissection, and mitral valve prolapse. The aortic pathologies are due to cystic medial necrosis of the aorta. Aortic dissection in Marfan's syndrome usually occurs in the ascending aorta and begins just superior to the coronary ostia. Patients will present with the classic signs and symptoms of aortic dissection, such as severe chest pain that radiates to the back, uneven pulses, or weak distal pulses.

Incorrect Answers:
Answer 1: Coronary atherosclerosis leading to myocardial infarction is the most common cause of sudden cardiac death in adults, but this patient’s young age, unusual phenotypic features, and family history of early death in his father makes a genetic syndrome more likely. Coronary atherosclerosis also would not explain this patient’s aortic regurgitation.

Answer 2: Hypertrophic cardiomyopathy (HCM) is a common cause of sudden cardiac death in young patients, but it usually does not present with pain. HCM would also not explain this patient’s phenotypic features or aortic regurgitation.

Answer 3: Spontaneous rupture of the aorta is not a known complication of Marfan's syndrome. Patients with Marfan's syndrome commonly present with aortic root dilation, aortic regurgitation, and aortic dissection.

Answer 4: Spontaneous rupture of the mitral chordae tendinae would cause severe mitral regurgitation. Although Marfan syndrome is associated with mitral valve prolapse and rupture of the chordae tendinae may occur, mitral regurgitation would cause a systolic murmur not heard in this patient.

Bullet Summary:
Patients with Marfan syndrome are at increased risk of aortic regurgitation and dissection due to cystic medial necrosis of the aorta.

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