A 24 year old tall thin man is evaluated for chest discomfort. He has long extremities, pectus excavatum, and lens subluxation. A decrescendo early diastolic murmur is heard at the left sternal border. Echocardiography shows dilation of the aortic root. Which of the following is the most likely underlying histopathologic change in the aortic media?
- A)Cystic medial degenerationGABARITO
- B)Calcific sclerosis
- C)Hyaline arteriolosclerosis
- D)Fibrinoid necrosis
- E)Fibromuscular hyperplasia
Explicação
Cystic medial degeneration is correct. Marfan syndrome due to fibrillin 1 mutation weakens the aortic media and predisposes to aortic root dilation, aortic regurgitation, and dissection. The diastolic murmur reflects aortic regurgitation from dilation of the r... Ver explicação completa e trilha adaptativa →