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Lethal enterovirus myocarditis associated with rituximab

Session Clinical Case Corner 4: When the heart is burning: cases of inflammatory cardiac involvement - Part I

Speaker Oscar Gonzalez Fernandez

Event : Heart Failure 2016

  • Topic : valvular, myocardial, pericardial, pulmonary, congenital heart disease
  • Sub-topic : Myocardial Disease – Clinical
  • Session type : Moderated Posters

O Gonzalez Fernandez1 , T Lopez Fernandez1 , C Alvarez Ortega1 , R Mori Junco1 , P Meras Colunga1 , V Rial Baston1 , J Irazusta Cordoba1 , E Lopez De Sa1 , M Moreno Yanguela1 , JL Lopez Sendon1 , 1University Hospital La Paz, Cardiology - Madrid - Spain ,

European Journal of Heart Failure Abstracts Supplement ( 2016 ) 18 ( Supplement 1 ), 301

Introduction: Myocarditis is an inflammatory disease of the myocardium with a wide range of clinical presentations, from subclinical disease to sudden death. Viral infection is the main aetiology and its treatment may require immune therapy when an unstable presentation occurs. Case report: A 39-year old white female without previous cardiovascular history was admitted to the coronary unit because of chest pain. A stage IVA follicular lymphoma was diagnosed six months before admission. The patient completed a first line regimen with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone. No completed remission was achieved after treatment, starting a second line regimen with rituximab, etoposide, methylprednisolone, cytarabine and cisplatin. The patient developed pericardial chest pain, hypotension and fever a few hours after the second cycle. On examination, blood pressure was 72/50 mmHg and heart rate was 92 bpm. Heart sounds were rhythmic without murmurs. An electrocardiogram showed an ST-segment elevation in the inferior leads and an echocardiogram was performed revealing a mild left ventricular systolic dysfunction with inferoseptal akinesia. Troponin I levels raised up to 50,1 ng/ml. A computerized tomography coronary angiogram was performed, showing normal coronary arteries. A cardiovascular magnetic resonance found the presence of late gadolinium enhancement involving the inferoseptal epicardium and mid myocardium. Left ventricular ejection fraction (LVEF) was 39%. PCR for detection of enterovirus was positive in pharyngeal and blood samples. According to these results an acute enterovirus myocarditis was suspected. After stabilizing the patient with inotropic agents, immune therapy consisting of intravenous methylprednisolone and immunoglobulin was initiated. A new echocardiogram revealed a deterioration of the LVEF down to 34% and severe functional mitral regurgitation. The patient developed dyspnoea, starting treatment with diuretics with an initial good response. Ten days after diagnosis the patient suffered a ventricular fibrillation, return of spontaneous circulation (ROSC) was achieved after thirty minutes of cardiopulmonary resuscitation. Cardiogenic shock resistant to inotropic agents and intraaortic balloon pump occurred and the patient was deceased after 48 hours. Discussion and conclusion: Acute myocarditis may associate in a minority of patients a poor prognosis. The differential diagnosis includes ischemic heart disease, valvular heart disease, congenital heart disease, other cardiomyopathies and pulmonary disease. Rituximab is a monoclonal antibody directed against the CD20 antigen on B-lymphocytes, which modifies the humoral immune response increasing viral infections. Lethal enterovirus myocarditis associated with rituximab is an extremely unusual condition described in the medical literature and its treatment should include haemodynamic support, immune therapy and mechanical support in unstable patients.

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