Abstract
Spontaneous coronary artery dissection (SCAD) is an infrequent cause of acute coronary
syndrome and is often underdiagnosed. Intramural hematoma is the most frequent angiographic
presentation and is a challenging diagnosis that may require intravascular imaging
techniques to confirm it and guide treatment. It affects mostly young women without
coronary risk factors and is usually associated with fibromuscular dysplasia. SCAD
has an underlying disease in 80% of patients. A SMAD3 mutation has been linked to aneurysm-osteoarthritis syndrome and has been identified
as a cause of familial thoracic aortic aneurysm and dissection. The first reported
case, to our knowledge, of a SMAD3 mutation underlying SCAD is described here.
Résumé
La dissection spontanée de l’artère coronaire (DSAC), une cause rare du syndrome coronarien
aigu, est souvent sous-diagnostiquée. L’hématome intramural est le signe angiographique
le plus fréquent ; son diagnostic difficile peut nécessiter des techniques d’imagerie
intravasculaire pour le confirmer et orienter le traitement. Il touche principalement
les jeunes femmes qui n’ont pas de facteurs de risque coronarien et est habituellement
associé à la dysplasie fibromusculaire. La DSAC dissimule une maladie sous-jacente
chez 80 % des patients. Une mutation SMAD3 a été associée au syndrome d’ostéoarthrite-anévrisme et a été considérée comme une
cause de la forme familiale d’anévrisme et de dissection de l’aorte thoracique. À notre connaissance, le cas décrit ci-après serait le premier cas déclaré d’une mutation SMAD3 sous-jacente à la DSAC.
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References
- Spontaneous coronary artery dissection: association with predisposing arteriopathies and precipitating stressors and cardiovascular outcomes.Circ Cardiovasc Interv. 2014; 7: 645-655
- Intracoronary imaging of coronary fibromuscular dysplasia with OCT and IVUS.Catheter Cardiovasc Interv. 2013; 82: E879-E883
- Spontaneous coronary artery dissection and its association with heritable connective tissue disorders.Heart. 2016; 102: 876-881
Arderiu G, Espinosa S, Peña E, Aledo R, Badimon L. PAR2-SMAD3 in microvascular endothelial cells is indispensable for vascular stability viatissue factor signaling. J Mol Cell Biol 2016;8:255-70.
- Exome sequencing identifies SMAD3 mutations as a cause of familial thoracic aortic aneurysm and dissection with intracranial and other arterial aneurysms.Circ Res. 2011; 109: 680-686
Article info
Publication history
Published online: September 24, 2016
Accepted:
September 12,
2016
Received:
April 11,
2016
Footnotes
See page 292.e3 for disclosure information.
Identification
Copyright
© 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
