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Dilated cardiomyopathy with severe arrhythmias in Emery-Dreifuss muscular dystrophy: from ablation to heart transplantation.


Summary. The male patient 38 years ago with 5 years has had muscle weakness, with 32 years arrhythmias. For 37 years he developed dilated cardiomyopathy, sick sinus syndrome, transient AV block II degree, paroxysmal atrial fibrillation, atrial flutter, ventricular arrhythmias. The examination revealed knees and elbows contractures, increased creatine kinase. DNA diagnostics revealed a frame shift deletion c.del619C in the gene EMD and c.IVS4-13T> A in the LMNA gene, and c.del619C deletion in the heterozygous state in a patient`s mother. RFA of cavotricuspid isthmus, ICD implantation were performed. Due to severe heart failure and electrical storm he underwent ECMO, heart transplantation nine months later. A morphological study revealed sclerosis, atrophy and hypertrophy of cardiomyocytes. After induction therapy (basiliximab) methylprednisolone, tacrolimus, mycophenolate were administrated. After 40 months follow-up the health status is satisfactory. Conclusion: heart failure in EDMD can progress rapidly unless the previously stable course; heart transplantation can be performed successfully using modern regimes of immunosuppression.

Credits: Olga Blagova, Alexander Nedostup, Dmitry Shumakov, Vitaly Poptsov, Anna Shestak, Elena Zaklyasminskaya



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