Spontaneous Coronary Artery Dissection After Pregnancy as First Manifestation of a Vascular Ehlers-Danlos Syndrome

J INVASIVE CARDIOL 2017;29(6):E67-E68.

Key words: Ehlers-Danlos syndrome, coronary artery dissection

A 43-year-old woman was admitted to our department for a troponin-positive acute coronary syndrome a few days after an uneventful pregnancy. An emergency coronary angiography showed a stenosis of the left anterior descending (LAD) and circumflex (CX) arteries that was not reversible with nitrates (Figure A). Due to the angiographic features suggestive of a coronary dissection, intracoronary imaging was employed. Optical coherence tomography (OCT) revealed intimal tears of dissection in the left main (LM; Figure B1) and proximal CX (Figure C2) with a wall hematoma in the mid LAD and CX (Figures D3 and E4). OCT highlighted the presence of red thrombus at the level of the dissection flap in the LM (Figure F1). We performed OCT-guided T-stenting of the CX and deployed a second drug-eluting stent from the ostial LM to the proximal LAD with final kissing-balloon inflation. Echocardiography, computed tomography at 3 months (Figure H), and coronary angiography at 9 months proved a good result, improvement of the non-stented segments, and a normal ejection fraction. Deeper investigations revealed vessel tortuosity and the unexpected finding of several pseudoaneurysmatic lesions of the internal carotid artery (Figure I) associated with a novel mutation in COL3A1 gene diagnostic for an aggressive vascular type of Ehlers-Danlos syndrome. Diagnosis, assessment, and treatment of a coronary dissection is highly contentious; a personalized approach based on clinical, angiographic, and coronary imaging is deemed necessary. Our invasive OCT-based strategy proved to be non-harmful and gave an excellent clinical result in the clinical context of a spontaneous multivessel coronary dissection with LM involvement in a rare connective tissue disorder. 

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From the Cardiovascular Department, Interventional Cardiology, Niguarda Ca’ Granda Hospital, Milan, Italy.

Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. The authors report no conflicts of interest regarding the content herein.

Manuscript submitted November 1, 2016 and accepted November 7, 2016.

Address for correspondence: Alberto Cereda, MD, Cardiovascular Department, Interventional Cardiology, Niguarda Ca’ Granda Hospital, Milan, Italy. Email: alberto.cereda@email.it