This case highlights that Danon disease can present with noncompaction cardiomyopathy and cause profound postoperative muscle weakness after heart transplantation, emphasizing the importance of early pathology results from the explanted heart.
Alerts clinicians to Danon disease in noncompaction cardiomyopathy before transplant; single case leaves open need for routine genetic evaluation.
INTRODUCTION: Postoperative muscle weakness is a serious complication in surgical intensive care patients. It is mostly described as critical illness polyneuromyopathy. Risk factors include intensive care length of stay, sepsis, poor glycemic control, and combined use of corticosteroids and neuromuscular blocking agents, malnutrition, and electrolyte imbalance. METHODS: We report a case of late-progressive, profound weakness after heart transplantation for noncompaction cardiomyopathy which required prolonged mechanical ventilation. The patient's muscle strength recovered completely after prolonged rehabilitation. RESULTS: Electromyographic assessment showed myopathy. Muscle biopsy revealed Danon disease, a genetic disorder affecting the lysosomal-associated membrane protein 2 gene (LAMP2). CONCLUSIONS: The finding of this genetic disorder was unexpected, because the preoperative echocardiographic diagnosis of noncompaction cardiomyopathy has not been reported in Danon disease. This report underlines the need for early availability of pathology results from the explanted heart, which showed the same disorder.
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Starre et al. (2012) studied this question.
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