Homozygous sickle cell disease (SCD) presents a multitude of challenges in patients undergoing cardiac surgery with cardiopulmonary bypass. Special consideration must be made in such patients and routine practice modified to prevent hypoxia, hypothermia, acidaemia and low-flow states which may potentially trigger a fatal sickling crisis perioperatively. We discuss several perioperative management strategies including a preoperative exchange transfusion, high flow normothermic bypass and warm blood cardioplegia that was utilized in a woman with homozygous SCD who underwent a successful double valve procedure.
Osteogenesis imperfecta is a connective tissue disorder that is rarely associated with isolated aortic insufficiency. Surgery on such patients carries high morbidity and mortality, which are mostly associated with bleeding tendencies secondary to increased tissue and capillary fragility. We report a 42-year-old male with isolated aortic incompetence, who underwent aortic valve replacement (AVR) with a mechanical prosthesis. The postoperative course was uneventful, and the patient was discharged home on the seventh postoperative day (POD 7). He remains well on follow-up 6 months later. We highlight the importance of a meticulous surgical technique, together with a strategy for management of anticipated perioperative complications to ensure a successful outcome.
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