Extend aortic repair for acute type A aortic dissection with rupture and malperfusion complicated with Ehlers-Danlos syndrome

Abstract

Background Ehlers-Danlos syndrome (EDS) is a disorder in the metabolism\nof fibrillary collagen. Its vascular type (vEDS) frequently develops\narterial rupture and aortic dissection. Case presentation The patient\nwas a 54-year-old gentleman who suffered from cardiac tamponade,\nmalperfusion of the left carotid artery and the right lower extremity\ndue to acute type A aortic dissection. Rupture of the aortic root and a\nhuge entry located from the transverse arch to the proximal descending\naorta were found. There were no significant deformities at aortic valve,\nso we tried extended repairs of valve sparing root replacement (VSRR)\nand total arch replacement (TAR) with frozen elephant trunk (FET) and\nwere successfully performed. He was finally diagnosed as vascular\nEhlers-Danlos syndrome (vEDS) by genetic examination. Conclusion\nEmergency extensive repairs of VSRR and TAR with FET for critical AAAD\nwith rupture and malperfusion was successfully performed for a vEDS\npatient.