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J Cardiovasc Med (Hagerstown). 2010 Aug;11(8):610-2. doi: 10.2459/JCM.0b013e3283314176.

Ascending aorta impending rupture: successful surgical management in a patient with type IV Ehlers-Danlos syndrome.

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  • 1Department of Cardiothoracic Surgery, St Antonius Ziekenhuis, Nieuwegein, The Netherlands.

Abstract

Ehlers-Danlos syndrome is an inherited disorder of the connective tissue characterized by increased elasticity of the skin, hypermobile joints and fragility of the vascular system. Vascular complications in these patients, requiring surgical repair, can be life-threatening with high perioperative morbidity and mortality. Reports of successful treatment of aortic ruptures or dissections in vascular Ehlers-Danlos syndrome patients are rare. We describe our surgical technique and precautions in an Ehlers-Danlos syndrome patient with a contained rupture of the ascending aorta, requiring emergency surgical intervention.

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