Myocarditis as a Manifestation of a T Cell Lymphoproliferative Disorder in a Patient Undergoing Left Ventricular Assistance Device Implantation

Abstract

<h3>Introduction</h3> Left ventricular assist device (LVAD) core pathology may provide definitive cardiomyopathy etiology and guide management. We present a case of progressive cardiomyopathy concerning for myocarditis in a patient with cutaneous T cell lymphoma (CTCL) undergoing LVAD implantation. <h3>Case Report</h3> A 63-year-old man presented with mildly reduced left ventricular ejection fraction (LVEF) and ventricular tachycardia (VT). He had an extensive history of Gulf War Syndrome, heavy metal exposure, chronic kidney disease, hypercalcemia and cutaneous T cell lymphoma, managed with ultraviolet light therapy and methotrexate. Cardiac magnetic resonance (CMR) imaging demonstrated anteroseptal and inferolateral wall delayed gadolinium enhancement (DGE). Coronary angiography showed no coronary artery disease. Fluorodeoxyglucose-positron emission tomography (FDG-PET) showed reduced lateral and inferior wall perfusion but no myocardial FDG uptake or evidence of systemic CTCL. Endomyocardial biopsy demonstrated mild hypertrophy and marked interstitial fibrosis, however no evidence of sarcoidosis or myocarditis. Over the next 2 years, his cardiomyopathy progressed (LVEF 15-20%, LVDd 7.2cm). Upon readmission for VT and heart failure (HF), repeat FDG-PET showed myocardial FDG uptake - perfusion mismatch pattern prompting oral prednisone initiation. Durable LVAD was implanted 5 days later given clinical progression. LVAD core pathology showed myocardial T cell infiltration with a clonal T cell gene rearrangement (Figure 1). Peripheral blood and PET markers remained otherwise negative for CTCL. After LVAD, his HF and VT stabilized, and he is undergoing treatment plan for systemic T cell lymphoproliferative disorder. <h3>Summary</h3> We present a case of myocardial T cell lymphoproliferative disorder presenting as progressive cardiomyopathy and highlight the role of LVAD core pathology in diagnosis.