Annals of cardiac anaesthesia
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Osteogenesis imperfecta is a rare disorder of connective tissues and presents multiple challenges, including difficult airway, hyperthermia, coagulopathy and respiratory dysfunction, for anesthesiologists, especially during cardiac surgery. We present anesthetic management of a patient with osteogenesis impertecta during double valve surgery. Dexmedetomidine infusion minimized the risks of malignant hyperthermia. ⋯ The mitral valve was replaced and the aortic valve repaired. Coagulopathy was corrected according to comprehensive coagulation analysis. Glidescope, dexmedetomidine, coagulation analysis and TEE could facilitate anesthetic management in these patients.
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Sudden cardiac arrest (SCA) in children is a rare, but catastrophic event. Children with cardiac pathology at particular risk include those with congenital long QT syndrome (CLQTS) and hypertrophic cardiomyopathy. ⋯ Use of an implantable cardioverter-defibrillator (ICD) is recommended for the prevention of SCA in this patient population. We report a case of CLQTS, who after successful resuscitation from SCA, underwent ICD placement at our center.