Document Type : Case Report
Authors
Department of Interventional Cardiology, San Giovanni Di Dio Hospital, Agrigento, Italy
Abstract
Background: Ehlers-Danlos syndrome (EDS) is a hereditary collagen vascular disorder characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. Vascular EDS (vEDS) is a subtype of EDS which is characterized by vascular fragility.
Case presentation: This is a case report of a young man with vEDS hospitalized for myocardial infarction. He was presented with a coronary dissection and developed aortic dissection, coronary rupture, and cardiac tamponade until death.
Conclusion: This case report highlights how patients with vEDS and acute coronary syndrome show a higher risk of vascular complications compared with other patients, and their admission to the institution with a cardiac surgery room could be helpful and safe for better management of the complications. Non-invasive methods could be useful to exclude other vascular diseases, before the emergency coronary intervention.
Highlights
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Keywords
2. Jorgensen A, Fagerheim T, Rand-Hendriksen S et al. Vascular Enhlers-Danlors Syndrome in siblings with biallelic COL3A1 sequence variants and marked clinical variability in the extended family. Eur J Hum Genet. 2015; 23 (6): 796-802.
3. Frank M, Adham S, Seigle S et al. Vascular Ehlers-Danlors Syndrome. Long term observational Study. J Am Coll Cardiol. 2019; 73 (15) 1948-1957.
4. Pepin M, Schwarze U, Superti-Furga et al. A Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med. 2000; 342: 673–680.
5. Adlam D, Alfonso F, Maas A, et al. European society of cardiology, acute cardiovascular care association, SCAD study group: a position paper on spontaneous coronary artery dissection. Eur Heart J. 2018; 39: 3353-3368.
6. Tweet MS, Hayes SN, Pitta SR et al. Clinical features, management, and prognosis of spontaneous coronary artery dissection. Circ J. 2012; 126: 579-588.
7. Hayes S, Kim ESH, Saw J et al. Spontaneous coronary artery dissection: current state of the science. Circ J. 2018;137:137-00.
8. Tweet MS, Eleid MF, Best PJ et al. Spontaneous coronary artery dissection: revascularization versus conservative therapy. Circ Cardiovasc Interv. 2014; 7 (6) :777–786.
9. Bergqvist D, Bjorck M, Wanhainen A. Treatment of vascular Ehlers- Danlos syndrome: a systematic review. Ann Surg. 2013; 258 (2): 257-26.
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