https://orcid.org/0000-0001-7251-2249
A 70-year-old woman with a previous cardiac pacemaker presented with breathlessness and orthopnoea. Electrocardiogram showed atrial fibrillation. A transthoracic echocardiogram revealed a right atrial mass. Cardiovascular magnetic resonance (CMR) imaging and subsequent positron emission tomography (PET) confirmed a large (11 cm × 8 cm) vascularized mass (Panels A and B, Supplementary data online, Video S1) infiltrating the right atrium, extending into the right ventricle and indenting the vena cava; with associated bilateral pleural effusions. Arrows highlight the abnormality (right atrial mass). The main differentials include angiosarcoma and lymphoma.
Histology from imaging guided biopsy confirmed diffuse large B-cell lymphoma (DLBCL). Following treatment with six courses of R-CHOP [Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Oncovin (vincristine), and Prednisolone] chemotherapy and two courses of intrathecal methotrexate, repeat imaging with CMR and PET (Panels C and D) demonstrated complete metabolic response to treatment.
Cardiac lymphomas consist of two main subtypes: mediastinal DLBCL invading the myocardium and primary cardiac lymphoma, which are confined to the heart and pericardium. Prognosis of DLBCL involving the heart has a very poor prognosis with a 10% survival rate in 9–12 months without treatment. Fortunately, lymphomas are generally highly responsive to treatment. Due to the limited number of cases and lack of standardized regime, treatment is case specific. The early post-chemotherapy period is critical due to potentially fatal complications, including ventricular fibrillation, refractory heart failure, massive pulmonary embolism, and cardiac rupture from rapid tumour destruction. Advanced multimodality imaging plays an important role in diagnosis, assessing for potential complications, and treatment response.
Supplementary data are available at European Heart Journal - Cardiovascular Imaging online.
