Feb 23, 2009 0
Syncope with a history of Marfan’s Disease…
A 53 yo woman collapsed in a crowd while celebrating inuaguration day on the mall in January of 2009. EMS was called by bystanders, who arrived to find the patient supine and in severe distress. She was able to reveal a medical history of Marfan’s disease, then became hypotensive and diaphoretic. She was immediately transported to the emergency department, where she was noted to be bradycadic, hypotensive, and unresponsive. She was intubated, central venous access was obtained, and a comprehensive workup including a chest and abdominal CT was performed. A bedside echocardiogram and abdominal ultrasound to evaluate the aorta were also performed. Initially no contrast was seen within the aorta, limiting the study for visualization of dissection or an intimal flap. However, upon repeat evaluation, the study reveals a complex dissection flap extending from the ascending aorta to bilateral iliac arteries, involving the L subclavian, common carotid, and renal artery. These bedside ultrasound images were obtained within the initial 20 minutes of the resuscitation:
Figure 1: Transverse view of the descending aorta. A low frequency, phased array probe is used for all images. Here the probe marker is oriented toward the patient’s R shoulder. Note the hyperechoic dissection flap seen in this cross-sectional view of the vessel.
Figure 2: Longitudinal view of the descending aorta. In this view the bright dissection flap is seen extending the length of the vessel at its posterior aspect.
Figure 3: A suprasternal view of the ascending aorta is obtained by placing the probe in the patient’s suprasternal notch, marker oriented straight up toward the patient’s head. The dissection flap is visible in this view as well, mandating surgical involvement.
Conclusion: The patient was taken emergently to the OR where she experienced complete hemodynamic collapse. Resuscitative efforts were unsuccessful, and she eventually died of complications of her disease.
Recommended reading: Bilku R, Steadman C, Jordan P. Acute deBakey type III (or Stanford type B) aortic dissection diagnosed by transthoracic
echocardiography. Journal of American Society of Echocardiography. 2008; 21(9): 1080-1083.
Special thanks to Dr.s Annette Dorfman and Jeremy Berman for their scanning skills and excellent clinical care!
