h STEOGENESIS IMPERFECTA (OI) is an inherited disease of the connective tissue.1-4 Because the incidence of I in the general population is low, the literature on manageent of patients undergoing cardiac surgery and particularly eep hypothermic circulatory arrest (DHCA) is scarce. The isorder of the collagen helix makes the connective tissues of eart valves, aorta, and capillaries very fragile.5-8 Redundant itral valve (MV) leaflets predispose for development of sysolic anterior motion (SAM) of the MV, which may be part of he connective tissue disturbance. The authors present a case eport of the general and cardiac anesthetic considerations of HCA in a patient with OI scheduled for coronary artery ypass graft surgery, aortic valve (AV) replacement, and asending aortic aneurysm repair associated with MV regurgitaion and SAM.