Canadian Journal of Cardiology

Electrical Storm in a Patient With Rosai–Dorfman Disease With Intracardiac Masses and Myocardial Infiltration

Published:March 06, 2020DOI:


      A 47-year-old woman with Rosai–Dorfman disease (RDD) presented with an electrical storm with sustained ventricular tachycardia. Echocardiography and computed tomography revealed an intracardiac mass involving the right atrium and left ventricle. Positron emission tomography demonstrated fluorodeoxyglucose avidity of the mass and myocardial infiltration; the electrical storm was attributed to this feature. Cardiac involvement in RDD is extraordinarily rare, and this appears to be the first report of an association of this disease with an electrical storm.


      Une femme de 47 ans atteinte de la maladie de Rosai-Dorfman (MRD) a présenté un orage rythmique avec tachycardie ventriculaire soutenue. Les examens par échocardiographie et par tomodensitométrie ont révélé une masse intracardiaque touchant l’oreillette droite et le ventricule gauche. La tomographie par émission de positrons a mis au jour l’avidité de cette masse pour le fluorodésoxyglucose ainsi qu’une infiltration du myocarde, laquelle expliquerait l’orage rythmique. Il est extrêmement rare que la MRD touche le cœur, et il semble qu’il s’agisse du premier cas d’orage rythmique signalé en lien avec cette maladie.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Canadian Journal of Cardiology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Abla O.
        • Jacobsen E.
        • Picarsic J.
        • et al.
        Consensus recommendations for the diagnosis and clinical management of Rosai–Dorfman–Destombes disease.
        Blood. 2018; 131: 2877-2890
        • O'Gallagher K.
        • Dancy L.
        • Sinha A.
        • Sado D.
        Rosai–Dorfman disease and the heart.
        Intractable Rare Dis Res. 2016; 5: 1-5
        • Sarraj A.
        • Zarra K.V.
        • Jimenez Borreguero L.J.
        • Caballero P.
        • Nuche J.M.
        Isolated cardiac involvement of Rosai-Dorfman disease.
        Ann Thorac Surg. 2012; 94: 2118-2120
        • Hendriks A.A.
        • Szili-Torok T.
        Editor’s Choice–The treatment of electrical storm: an educational review.
        Eur Heart J Acute Cardiovasc Care. 2018; 7: 478-483
        • Summers M.R.
        • Pettersson G.
        • Maalouf J.F.
        • Jaber W.A.
        Sinus histiocytosis with massive lymphadenopathy: extra-nodal Rosai-Dorfman disease presenting as a rare aetiology of a large intracardiac mass.
        Eur Heart J. 2017; 38: 1439-1440