Case report
In 2017, a 52-year-old man was successfully transplanted for acute advanced heart failure due to massive myocardial infarction, going through a step of left ventricular assistance device complicated by thromboembolic accident. Serology was mismatched, the donor was EBV positive and the patient EBV negative. After transplant no adverse event occur except an moderate chronic renal failure. For protect the renal function we treated him by prednisolone, mycophénolate mofétil, éverolimus and ciclosporine in order to use low dose of calcineurine inhibitor. In December 2019, he was admitted to our hospital for severe but well-tolerated palpitations. The ECG showed a ventricular tachycardia (Fig.1) that was reduced by beta blocker (Atenolol ®). Echocardiographic parameters showed no myocardial dysfunction and no sign of graft rejection. Further investigations did not bring any biological abnormalities beside an Epstein-Barr Virus (EBV) high replication - 30 4000 IU/ml. Cardiac computed tomography and cardiac magnetic resonance imaging revealed two nodular images developed in the antero-medium and apical wall of the right ventricle and extending intracavitary and to epicardial area (Fig. 1). Endomyocardial biopsies were performed and ruled out an acute rejection. Nonetheless, the microscopic appearance revealed a post-transplantation monomorphic lymphoproliferative disorder, EBV induced, high grade B cell lymphoma type Diffuse large B-cell lymphoma (DLBCL). As illustrated in Figure 1, the 18FDG-PET indicated an important lymphoma extension with three hypermetabolic pericardial tumors. Of note, a coronary angiography ruled out an additionnaly underlying ischemic cardiomyopathy but hypervascularization was observed in the intramyocardial tumoral masses. We proceed with an initial therapy of four cycles of rituximab and a reduction of the immunosuppressive drug in order to decrease the EBV replication. Unfortunately, despite pursing beta-blockers treatment ventricular arrhythmias still occurred including ventricular premature beats and sustained ventricular tachycardia (VT). Due to the sporadic nature of the arrhythmia, we decided not to implant an implantable cardioverter-defibrillator (ICD) as the first line therapy but to optimize the antiarrhythmic therapy by inflating beta-blockers to the maximum tolerated dose. Despite optimal medical therapy including amiodarone and beta-blockers, the patient presented an electrical storm associated with hemodynamic instability and was hospitalized in the Cardiac Intensive Care Unit (CICU). In CICU, the VT recurrences were controlled using Esmolol infusion. After 2 days, the patient was free from VT recurrence and Nadolol was introduced with a good tolerance, authorizing the continuation of the chemotherapy. Fourth cycles of Rituximab were consequently completed and a PET reevaluation was realized showing the persistence of an intense hypermetabolism in the three pericardial tumors. A new therapy using Doxorubicin, Cyclophosphamide and Vincristine was implemented as an emergency treatment with regular PET reevaluations and a minimized immunosuppression. In order to prevent the sudden cardiac death, the patient was implanted with an endocavitary ICD (Medtronic, Dublin, ireland). The first cycle of chemotherapy caused a pancytopenia with a profound thrombocytopenia resulting in the formation of an ICD pocket hematoma. The hematoma spontaneously progressed to fistula, leading to a revision surgery with device removal. Before hospital discharge, the patient received a life-vest (Zoll, Chelmsford, USA) that was badly tolerated. Successive chemotherapy cycles were ineffective with a tachyphylaxis situation and a quick progression of the tumoral lesions assessed by cardiac imaging and significant EBV replication. The patient died from a sudden death at home 3 month after the diagnosis, not wearing his life-vest anymore because of the bone pain caused by chemotherapy.