Occlusion of a coronary artery by an acute type A aortic dissection presents a life-threatening emergency that is rarely seen and easy to misdiagnose. We present the case of a 75-year-old male who experienced sudden onset of severe left-sided chest pain due to an acute type A aortic dissection that obstructed the right coronary artery. Following an initial misdiagnosis of acute coronary syndrome, imaging revealed the presence of an aortic dissection. An emergency modified Bentall procedure was performed, in which the damaged aorta and aortic valve were replaced.
Background Ehlers-Danlos syndrome (EDS) is a disorder in the metabolism
of fibrillary collagen. Its vascular type (vEDS) frequently develops
arterial rupture and aortic dissection. Case presentation The patient
was a 54-year-old gentleman who suffered from cardiac tamponade,
malperfusion of the left carotid artery and the right lower extremity
due to acute type A aortic dissection. Rupture of the aortic root and a
huge entry located from the transverse arch to the proximal descending
aorta were found. There were no significant deformities at aortic valve,
so we tried extended repairs of valve sparing root replacement (VSRR)
and total arch replacement (TAR) with frozen elephant trunk (FET) and
were successfully performed. He was finally diagnosed as vascular
Ehlers-Danlos syndrome (vEDS) by genetic examination. Conclusion
Emergency extensive repairs of VSRR and TAR with FET for critical AAAD
with rupture and malperfusion was successfully performed for a vEDS
patient.
Staged Hybrid Repairs of Acute Type A Aortic Dissection Involving the Right Aortic Arch with Retroesophageal Aortic Segment