Acute myopathy of intensive care in a child after heart transplantation

Purpose: Acute myopathy of intensive care has been described infrequently i n children and never after organ transplantation. We report a case of acute myopathy of intensive care in a child after heart transplantation. Clinical features: An 11-yr-old girl, with no previous medical history, dev eloped acute cardiomyopathy leading to cardiac shock. Family history reveal ed four cases of unidentified myopathy and/or cardiomyopathy, Preoperativel y, while muscle biopsy was near normal, myocardial biopsy revealed non spec ific mitochondrial disorders. A few days after heart transplantation, she d eveloped acute hypotonia and flaccid quadriplegia, consistent with the diag nosis of acute myopathy of intensive care. Nerve conduction studies were no rmal, electromyography showed myopathic changes and a new muscle biopsy fro m quadriceps femoris showed severe loss of myosin filaments and ATPase acti vity in type 2 fibres, A large laboratory screening failed to demonstrate a metabolic disease or a known myopathy. Muscle strength recovered progressi vely in three weeks allowing home discharge, A few months later, she was fr ee of symptoms and muscle biopsy showed full histopathological recovery, Conclusion: Acute myopathy of intensive care can occur in children after he art transplantation. It should be suspected in the presence of muscle weakn ess and difficulty in weaning from ventilatory support. Electromyography co nfirmed a myogenic process and muscle biopsy allowed diagnosis. Full clinic al and histopathological recovery usually occur within three weeks.