Key result
Transapical aortic cannulation in Ehlers-Danlos Syndrome Type IV leads to LV pseudo-aneurysm requiring reoperation.
Why the study?
Ehlers-Danlos syndrome may cause vulnerability of cardiac muscle and suture dehiscence after transapical aortic cannulation, but clinical experience is limited.
Case Report (n=1)
No
Transapical cannulation should be used with extreme caution in patients with Ehlers-Danlos syndrome due to the high risk of tissue vulnerability, suture dehiscence, and pseudo-aneurysm formation.
Caution advised for transapical cannulation in Ehlers-Danlos syndrome type IV; leaves open optimal aortic access in connective tissue disorders.
Introduction:We report a case of Ehlers-Donlos syndrome which raised left ventricular pseudo-aneurism at the site of transapical aortic cannulation after acute aortic dissection repair.Case Report: A 49-year-old male underwent total arch replacement for type A aortic dissection using transapical aortic cannulation.CT revealed a pseudoaneurysm at the left ventricular apex 2 weeks after the initial surgery, and the size of pseudoaneurysm increased in another 2 weeks.We performed reoperation and found suture dehiscence of transapical cannulation closure.Post-operative course was uneventful.He was diagnosed as Ehlers-Donlos syndrome by genetic examination after the second surgery. Conclusion:Ehlers-Danlos syndrome might be attributed to vulnerability of cardiac muscle and suture dehiscence, and transapical cannulation should be carefully applied in the patients with Ehlers-Danlos syndrome.
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Takano et al. (2015) conducted a case report in Ehlers-Danlos Syndrome Type IV and Acute Aortic Dissection (n=1). Transapical Aortic Cannulation was evaluated. Transapical aortic cannulation in a 49-year-old man with Ehlers-Danlos Syndrome Type IV resulted in a left ventricular pseudo-aneurysm due to suture dehiscence requiring reoperation.
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