This case report describes a previously undescribed association between osteogenesis imperfecta and type I aortic dissection, highlighting a fatal complication of re-dissection post-surgical repair.
Warrants vigilance for coronary re-dissection in osteogenesis imperfecta patients after aortic repair; leaves open optimal surgical strategies for this rare association.
The previously undescribed association between aortic dissection and osteogenesis imperfecta is reported in a 39-year-old man with known osteogenesis imperfecta, who presented with a type I aortic dissection. His ascending aorta was repaired using a Dacron tube graft under hypothermic circulatory arrest but the patient died 12 h later, following re-dissection around the left coronary ostium resulting in massive myocardial infarction of the left ventricle.
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Ashraf et al. (1993) studied this question.
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