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.