Osteogenesis imperfecta (OI) is an inherited connective tissue disorder, a
group that includes Ehlers-Danlos syndrome, Marfan's syndrome and pseudoxan
thoma elasticum. OI is a heterogeneous disease of collagen I biosynthesis c
haracterized by variable clinical phenotypes, including skeletal and cardio
vascular manifestations. A 65-year-old man with OI who had extensive prior
successful cardiac valve surgeries is described. He survived for 18 years a
fter his initial valve surgery, but died of multiorgan failure and sepsis a
fter repair of a spontaneous type A aortic dissection. This is the fourth r
eported case of aortic dissection secondary to OI and illustrates the exten
sive cardiovascular pathology associated with OI. Aggressive management of
arterial dissection risk factors, such as systemic arterial hypertension, i
s advocated for patients with OI.