Recurrent ventricular fibrillation in a patient with a cardiac mass

Robert Römer (Jena)1, L. Reinhardt (Jena)1, J. Hammersen (Jena)2, R. Pfeifer (Jena)1, C. Schulze (Jena)1, M. Fritzenwanger (Jena)1

1Universitätsklinikum Jena Klinik für Innere Medizin I - Kardiologie Jena, Deutschland; 2Universitätsklinik Jena Klinik für Innere Medizin II Abteilung Hämatologie und Internistische Onkologie Jena, Deutschland


Recurrent ventricular fibrillation in a patient with a cardiac mass
Robert Römer, Lisa Reinhardt, Jakob Hammersen, Rüdiger Pfeifer, Paul Christian Schulze, Michael Fritzenwanger
Internistische Intensivstation, Klinik für Innere Medizin I, Universitätsklinikum Jena, Am Klinikum 1, 07747 Jena
Cardiac tumors are rare and non-Hodgkin lymphomas represent the rarest subgroup. Cardiac infiltration usually occurs in the right atrium or ventricle. Cardiac lymphomas have an extremely poor prognosis if left untreated. With adequate and timely therapy, remission and a significant improvement in the prognosis are possible.
We report on a 72-year-old patient who presented to our institution in March 2023 due to recurrent fever and increased infectious parameters. A focus search was carried out, which remained unremarkable. However, syncope occurred twice with self-limiting tachycardia, so she was transferred to the monitoring station. There were several episodes of ventricular fibrillation with subsequent resuscitation. Coronary heart disease was ruled out. Because of a prior total femur replacement after a periprosthetic fracture, a PET-CT was performed to exclude a prosthesis infection.
PET-CT showed masses in the left and right ventricles (Pic. 3, 4), the left kidney and adrenal gland, the small intestine and a supraclavicular lymph node. Imaging using transthoracic echocardiography was performed. This showed a smaller hyperechoic mass in the area of the right ventricle, as well as another mass on the lateral wall of the left ventricle, measuring 9.5 by 3.3 cm (Pic. 1, 2). For histological differentiation, a transcatheter biopsy of the left ventricular mass was performed. 
The histological workup revealed a diffuse large-cell B-cell lymphoma (CD20+, PAX5+, CD30-) infiltrating the myocardium with high proliferation rate (Ki67 at 80%). The masses seen on PET-CT in the left kidney and adrenal gland, the small intestine and a supraclavicular lymph node were described as extracardiac lymphoma manifestations. The patient received preliminary phase therapy with prednisolone, followed by cyclophosphamide analogous to the Burkitt protocol, doxorubicin and rituximab (R-CHOP) for a total of 6 courses. 
Intermittent ventricular fibrillation occurred during the preliminary phase therapy, so antiarrhythmic therapy with amiodarone was initiated. Ventricular fibrillation recurred during the first course of R-CHOP, an electrical device therapy was discussed. An implantable cardioverter-defibrillator was not feasible for right ventricular tumor infiltration, nor was a defibrillator vest feasible for polyneuropathy, so a subcutaneous implantable cardioverter-defibrillator was implanted. After the second course of R-CHOP, echocardiography showed a clear regression of the intracardiac tumor mass. No new arrhythmias were evident in the ICD check-ups. After completing the 6th course, follow-up monitoring using PET-CT showed complete remission of both cardiac and extracardiac lymphoma manifestations.
Cardiac lymphomas with cardiac involvement are a rare cause of recurrent ventricular fibrillation. Remission of the neoplasm can be achieved using chemoimmunotherapy. The ventricular fibrillation was reversible after remission was achieved. Cardiac lymphomas present a particular challenge when selecting device therapy.
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