17yo boy with long legs and arms, h/o scoliosis and mild pectus excavatum, hyperextensibility of the thumbs, presents with sudden onset ripping quality chest pain and feeling lightheaded. On exam, patient is anxious appearing, afebrile, HR 105, BP 98/45, RR 14, pulse ox 98% RA, alert, HEENT non-revealing, Lungs BCTA, Cor RRR with mid-systolic click followed by a late systolic murmur at the apex, Abd soft ND NT, Extremities and Skin non-contributory. Of the following, which diagnostic study is the best choice?
A) POC troponin
B) Arterial blood gas
C) Bedside ultrasound looking for lung sliding
D) CT angiogram
E) CT abdomen
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August 10, 2016 at 12:02 pm
CT angio – dissection with extension to valve.
August 10, 2016 at 9:13 pm
Answer: D (CT angio, as Lacey said)
The patient presented has physical characteristics of Marfan syndrome, including mitral valve prolapse. The presentation is worrisome for aortic dissection. The patient is hemodynamically stable, so CT or MRI angiogram are good diagnostic studies, although the study of choice if available would be transesophageal echocardiogram (TEE). An initial CXR to assess for widened mediastinum and comparison of the BP’s between right and left arm may also be helpful in making/suspecting the diagnosis.
August 10, 2016 at 9:30 pm
Also, here is a great quick read from Pediatric EM Morsels http://pedemmorsels.com/marfan-syndrome/