Is the epicardial left ventricular lead implantation an alternative approach to percutaneous attempt in patients with Steinert disease? A case report

Andrea Antonio Papa 1,2, Anna Rago 1, Roberta Petillo 2, Paola D’Ambrosio 2, Marianna Scutifero 2, Marisa De Feo 3, Ciro Maiello 4 and Alberto Palladino 2

1Department of Cardiothoracic Sciences, Chair of Cardiology, Monaldi Hospital, University of Campania “L. Vanvitelli”; 2Cardiomyology and Medical Genetics, University Hospital of Campania “L. Vanvitelli”; 3 Department of Cardiothoracic Sciences, Unit of Cardiac Surgery, Monaldi Hospital; 4 Transplant Surgery Unit, AORN Ospedali dei Colli, Monaldi Hospital, Naples, Italy

Steinert’s disease or Myotonic Dystrophy type 1 (DM1) is an autosomal dominant multisystemic disorder characterized by myotonia, muscle and facial weakness, cataracts, cognitive, endocrine and gastrointestinal involvement, and cardiac conduction abnormalities. Although mild myocardial dysfunction may be detected in this syndrome with age, overt myocardial dysfunction with heart failure is not frequent. Cardiac resynchronization therapy is an effective treatment to improve morbidity and reduce mortality in patients with DM1 showing intra-ventricular conduction delay and/or congestive heart failure. We report the case of a patient with Steinert disease showing an early onset ventricular dysfunction due to chronic right ventricular apical pacing, in which an epicardial left ventricular lead implantation was performed following the failure of the percutaneous attempt. As no relief in symptoms of heart failure, nor an improvement of left ventricular ejection fraction and reverse remodelling was observed six months later, the patient was addressed to the heart transplantation. 

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