Multiparametric cardiac magnetic resonance imaging (CMR) for the diagnosis of Loeffler’s endocarditis: a case report
© The Author(s). 2017
Received: 7 September 2016
Accepted: 3 February 2017
Published: 11 March 2017
Endocarditis parietalis fibroplastica Löfflein (EPF) is a rare form of primary restrictive cardiomyopathy with poor prognosis. It is generally caused by hypereosinophilic syndrome with eosinophilic penetration of the heart. This leads to congestive heart failure in three different stages. As a frequent manifestation of neoplastic diseases, cardiac involvement means poor prognosis.
The present report deals with a case of EPF caused by non-specified T-cell lymphoma (T-NOS). Besides an elevated Troponin-T enzyme, the electrocardiogram and the transthoracic echocardiography did not show any characteristic results. Due to risk/benefit assessment and low thrombocyte amounts, endomyocardial biopsy and catheterization were discarded. Using cardiovascular magnetic resonance (CMR) with steady-state free precession sequences, T2-mappping, strain analysis and late gadolinium enhancement, we were able to clearly highlight cardiac involvement at different stages. These findings characterized T-NOS as a palliative situation.
Multiparametric CMR can not only identify EPF but also characterize the patchy disease state. This provides an individual prognosis assessment. Aside from prognosis estimation, it can also be used for therapy monitoring.
KeywordsCMR Restrictive cardiomyopathy Loeffler’s endocarditis Heart failure Hypereosinophilic syndrome Fibrosis Case report
Endocarditis parietalis fibroplastica (Loeffler’s disease) (EPF) is a disease with poor prognosis, first described by Loeffler in 1936. A diagnosis requires blood eosinophilia and affection of the endocardium as key steps . The EPF belongs to the hypereosinophilic syndromes (HES) with rare epidemiologic data. Spry et al. showed a prevalence of 1:200000 in general population . In more recent studies, the prevalence of EPF is shown from 40 to 50% among patients with HES . Eosinophils must be elevated over 1.5x109 eosinophils per litre for about 6 months to lead to the diagnosis of hypereosinophilia, although blood levels may lack hypereosinophilia in later stages. HES syndrome can be idiopathic or correlated to clonal interleukin 5 (IL-5) over-production of T-helper cells. Once the heart is affected, the disease passes three steps: 1. Acute necrosis followed by 2. thrombosis and 3. end stage of fibrosis . The underlying pathophysiology is a degradation of cytotoxic eosinophilic proteins, which may reveal endocardial thickening, ventricular obliteration by echogenic material, suggestive of fibrosis or thrombosis, atrial dilation, restrictive pattern in echo Doppler or coronary vessel alterations . Still, diagnostic process is heterogeneous and is yet to be standardized. Electrocardiogram (ECG) and transthoracic echocardiography (TTE) are unspecific and these technologies have certain restrictions (i.e. insufficient acoustic window). Endomyocardial biopsy is supposed to be the gold standard of diagnostic tools, but exerts a considerable inherent sampling error and may thus suffer from insufficient diagnostic sensitivity. Cardiovascular magnetic resonance (CMR) might be able to characterize the specific stages of the disease due to its superior native soft tissue contrast and further tissue characterisation by contrast-enhanced sequences.
Diagnosis of non-specified T-cell lymphoma (T-NOS); Stage III B Ann Arbor
12/12 – 03/13:
Massive progress of T-NOS (change from CHOEP to DHA, to vincristine and SDH in emergency)
Autogenic stem cell transplantation + Radiochemotheraphy (Cyclophosphamid)
Allogenic stem cell transplantation
Thrombosis Vena cava inferior/iliaca communis
Remission of T-NOS
Relapse of T-NOS
07/15 – 01/16:
IGEV-Therapy, bendamustine, DLI
Change to everolimus
Diagnosis of Loeffler’s endocarditis
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.
Discussion and Conclusion
The present case of EPF illustrates that multiparametric CMR can not only identify EPF, but also characterize EPF according to the disease state. CMR is a highly efficient tool for the process of diagnosis if invasive procedures are discarded due to risk/benefit assessment. In this particular case, myocarditis and coronary artery disease could be excluded without EMB or angiography due to multiparametric CMR. In addition, CMR detected advanced endocardial fibrosis with wall thickening as morphological hallmarks of the disease, while those features would have technically impeded adequate EMB.
Cardiac magnetic resonance
Endocarditis parietalis fibroplastica Löfflein
Global longitudinal strain
Late gadolinium enhancement
Steady state free precession
Non-specified T-cell lymphoma
No funding has been received.
Availability of data and materials
All data generated or analyzed during this case report are included in this published article.
MG and FB have been involved in drafting the manuscript, have made substantial contributions to conception and presentation of the case and have revisited it critically for important intellectual content. They have given final approval of the version to be published. CJ has made substantial contributions to conception and design of the case. Together with PB and MK, they have been involved in revising the manuscript critically for important intellectual content and have given final approval of the version to be published.
The authors declare that they have no competing interests.
Consent for publication
Written informed consent was obtained from the patient for publication of this case report. A copy of the written consent is available for review by the corresponding author.
Ethics approval and consent to participate
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