A 38-year-old man with a medical history of non-specified T-cell lymphoma (T-NOS) including immunosuppressive therapy regimes and a stem cell transplantation came to the emergency unit of the university hospital in Duesseldorf because of his uncontrolled nose bleeding (Table 1). Next to low thrombocyte amounts (less than 9000/μl), blood diagnostic showed an elevation of the Troponin-T enzyme up to 324 ng/l (normal cut-off at 14 ng/l). ECG revealed unspecific negative T-waves in leads II, III, aVF, V4 and V6. In TTE, only a slight hypertrophy of the interventricular septum and the left ventricular posterior wall was detected, while the global ventricular function was normal. Due to inadequate acoustic window, the quality of TTE images proved too poor for strain evaluation. The patient was transferred to the department of cardiology on suspicion of an acute myocarditis or coronary artery disease. Due to risk assessment (GRACE Score <140) and thrombocytopenia, invasive coronary angiography or endomyocardial biopsy (EMB) were initially discarded. In the meantime, a CMR examination for identification of myocardial lesions was conducted.
Cine steady state free precession (SSFP) images revealed normal left and right ventricular function (LV ejection fraction (EF) 61%, RV-EF 61%). As can be seen in Fig. 1a (doubled arrows), LV and RV walls were thickened and displayed subendocardial susceptibility artefacts (arrow). Despite normal LV-EF, myocardial feature tracking analysis (TomTec Imaging Systems, Unterschleißheim, Germany) revealed impaired systolic endocardial and epicardial global longitudinal strain (GLS) compared to literature standards (-17.7% vs. -22.2% for endocardial GLS; -14.5% vs. -20.4% for epicardial GLS) [6]. Peak early diastolic strain rate (SRe) was impaired in epi- and endocardium as well, indicating diastolic impairment (1.2 s-1 vs. 1.7 s-1 for endocardial diastolic SRe; 1.0 s-1 vs. 1.5 s-1 for epicardial diastolic SRe) (Fig. 1b).
Perfusion images showed an imbalance of transmyocardial perfusion already under resting conditions (Fig. 2a, arrows). This phenomenon was aggravated during regadenoson induced hyperaemia, that showed highly impaired perfusion in endocardial layers, indicating altered coronary microcirculation (Fig. 2b).
To analyse myocardial tissue texture, a parametric CMR was conducted to separate oedema or acute necrosis to other tissue texture components with T2 mapping [7]. As shown in Fig. 3a, T2 mapping revealed a slightly reduced T2 time (55.0 ± 8.4 ms, arrows) compared to an age- and gender-matched control (56.4 ± 3.5), which was still in the normal value range according to current literature [8]. Epimyocardial T2 time was elevated compared to the control (69.6 ± 11.9 ms vs. 60.3 ± 4.6 ms, arrowheads) suspecting acute tissue oedema [8]. Diffuse subendocardial late gadolinium enhancement (LGE) was detected in both chambers in those areas of slightly reduced T2 time completing the previous findings (Fig. 3b, arrows).
Taking the localisation of fibrosis, the T2 map and the perfusion abnormalities into account, the hypotheses of vascular coronary disease or myocarditis were discarded since there was neither an endocardial lesion corresponding to a coronary territory nor an epicardial lesion. Additional details of the patient’s history revealed a hypereosinophilia (max. 11.900/μl, normal value < 440/μl) for more than 6 months. On the basis of the CMR findings and the patient’s history of T-NOS with hypereosinophilia, he was diagnosed to have Endocarditis parietalis fibroplastica Löfflein (EPF). Interestingly, the multiparametric CMR workup revealed different stages of myocardial involvement: reduced GLS, diffuse LGE, perfusion defect and slightly lower T2-time displayed fibrosis and reduced coronary flow of the endocardium already under resting conditions. The epicardium, however, was characterized by abnormal GLS with abnormal diastolic function, lack of LGE, preserved perfusion, but elevated T2 times suggesting an inflammatory stadium. A high-dose corticoid drug (Dexamethasone 40 mg) was added to the existing everolimus-therapy and the patient was transferred to the department of haematology for further diagnostics. His bone marrow showed elevated numbers of eosinophilia up to 10% during completion of diagnostic steps.
Cardiac involvement at different stages of EPF as shown by multiparametric CMR characterized T-NOS as a palliative situation, as recent therapeutic regimes such as immunosuppressive drugs and stem cell transplantation did not lead to a lasting remission.
Since multiparametric CMR workup could not only identify EPF but also characterize the patchy disease state (T2 map, LGE and myocardial strain) and stratify the patient’s individual prognosis, invasive coronary angiography and endomyocardial biopsy were not conducted.
