Fatal Aortic Rupture Presenting as Chest Pain in an Adolescent: The Role of Echocardiography in Occult Cystic Medial Necrosis

An adolescent female with occult cystic medial necrosis died following spon taneous aortic rupture. A large saccular aortic aneurysm that had ruptured into the pericardial space was demonstrated by two-dimensional echocardiography and confirmed at surgery. Echocardiographic screening of the patient&...

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Bibliographic Details
Published in:Clinical pediatrics 1985-04, Vol.24 (4), p.216-218
Main Authors: Schaffer, Michael S., Nouri, Soraya, Chen, Su-chiung, Waggoner, Alan D., Pennington, D. Glenn, Monteleone, Patricia L.
Format: Article
Language:English
Subjects:
Adolescent
Aorta, Thoracic
Aortic Diseases - complications
Aortic Diseases - genetics
Aortic Rupture - diagnosis
Aortic Rupture - etiology
Diagnosis, Differential
Echocardiography
Female
Humans
Male
Necrosis
Pain - etiology
Thorax
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Summary:An adolescent female with occult cystic medial necrosis died following spon taneous aortic rupture. A large saccular aortic aneurysm that had ruptured into the pericardial space was demonstrated by two-dimensional echocardiography and confirmed at surgery. Echocardiographic screening of the patient's family members revealed a 13-year-old brother with unsuspected aortic root dilatation. He is now being followed for possible progression of his disease. This case demonstrates the role of echocardiography in cystic medial necrosis. It can aid the acute management of patients with aortic dissection or aneurysm. It can also define patients with occult disease who require serial follow-up and genetic counseling.
ISSN:0009-9228
1938-2707
DOI:10.1177/000992288502400408