Keywords
aortic dissection - tamponade - Loeys–Dietz syndrome
A 16-year-old boy experienced a sudden loss of consciousness at home in front of his
parents. On arrival, he was in cardiac arrest with pulseless electrical activity.
An ultrasound study revealed a high-echoic layer surrounding the heart with a high-echoic
layer surrounding the ascending aorta as well ([Figs. 1] and [2]). He received a diagnosis of clotting cardiac tamponade. Urgent thoracotomy with
pericardiotomy was performed ([Fig. 3]), but he failed to obtain return of spontaneous circulation. Autopsy imaging indicated
residual pericardiac hematoma ([Fig. 4]). Based on his features, he was suspected of having Loeys–Dietz or Marfan syndrome.
We postulated that connective tissue disease had induced Type A aortic dissection
with subsequent rupture that had resulted in cardiac tamponade and cardiac arrest.
Permission to perform a genetic analysis was not obtained from his parents.
Fig. 1 The ultrasound study revealed a high-echoic layer (arrow) surrounding the heart (arrowheads)
suggesting clotting cardiac tamponade.
Fig. 2 The ultrasound study revealed a high-echoic layer (arrow) surrounding the ascending
aorta, suggesting aortic dissection.
Fig. 3 Urgent thoracotomy with pericardiotomy revealed a clot weighing approximately 1 kg.
Fig. 4 Autopsy imaging. Computed tomography indicated residual pericardiac hematoma.
In cases of hemorrhaging into a closed space, the blood forms a blood clot to achieve
hemostasis. During this process, the whole blood separates into a blood clot and serum.
When the serum accumulates in one space, it may be detected as fluid by ultrasound.
However, it takes some time for the serum to accumulate. Accordingly, a focused assessment
with sonography in trauma, which focuses on low-echoic areas to detect serum, is well
known to have a high false-negative rate for the acute hemorrhaging state in traumatized
patients.[1] Clots are scanned as high-echoic areas on ultrasound studies. If physicians focus
not only on low-echoic but also high-echoic areas, the sensitivity for detecting clinically
significant blood clots in the human body may be improved.
Unfortunately, the present patient failed to obtain a favorable outcome; however,
the urgent resolution of cardiac tamponade by clotting and subsequent treatments might
result in a favorable outcome in other patients.[2]
[3]
