European Heart Journal - Case Reports (2021) 5(11), 1–2

CARDIOVASCULAR FLASHLIGHT
Cardiac imaging

doi:10.1093/ehjcr/ytab298

Fulminant cardiac and renal sarcoidosis
revealed by electron microscope: challenging
aspect of diagnosis
Seiichiro Naito
Toshihisa Anzai, Shingo Tsujinaga*, Kiwamu Kamiya, Toshiyuki Nagai, andDepartment of Cardiovascular Medicine, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Kita-15, Nishi-7, Kita-ku, Sapporo 060-8638, Japan
Received 14 June 2021; first decision 1 July 2021; accepted 20 July 2021; online publish-ahead-of-print 28 July 2021

* Corresponding author. Tel: þ81-11-706-5755, Fax: þ81-11-706-6973, Email: shingo-t.0207@med.hokudai.ac.jp
Handling Editor: Rita Pavasani
C The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology.
V
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2S. Naito et al.

Video 1 Echocardiography in parasternal long-axis view.
Video 2 Echocardiography in 4-chamber view.
A 62-year-old woman with no prior history of cardiovascular andrenal disease was referred to our department due to progressive cardiorenal dysfunction with fatigue and dyspnoea over the past
3 weeks. Chest X-ray revealed lung congestion and electrocardiogram showed intraventricular conduction disturbance (Supplementary material online, Figure S1). The laboratory tests revealed anincreased level of serum N-terminal-pro-B-type natriuretic peptide(72 862 pg/mL) (reference value < 55 pg/mL), troponin T (466 ng/L)(reference value < 16 ng/L), creatinine (5.53 mg/dL) (reference value
0.65–1.07 mg/dL), angiotensin-converting enzyme (ACE, 26 U/L)
(reference value 8.3-21.4 U/L), and soluble interleukin-2 receptor(s-IL2R, 6151 U/mL) (reference value < 613 U/mL). Echocardiography showed severely reduced left ventricular ejection fraction(LVEF) (25%) without basal thinning of interventricular septum,
which is characteristic feature of cardiac sarcoidosis, or wall thickening as seen in acute infective myocarditis (Panel A, Videos 1 and 2).
Coronary angiogram excluded coronary artery disease, andlight-microscopy of endomyocardial biopsy specimen from the rightventricular septum did not show any findings of sarcoidosis or myocarditis (Panel B). The haemodynamic condition progressively worsened, resulting in the implantation of a intra-aortic balloon pump andextracorporeal membrane oxygenation. On the basis of elevated
ACE and s-IL2R levels, we suspected the existence of fulminant cardiac and renal sarcoidosis and decided to administrate pulse methylprednisolone therapy (1000 mg/day for 3 days) followed by amaintenance dose of prednisone (started from 40 mg/day and gradually tapered to 5 mg/day). Her haemodynamics dramatically
improved thereafter, and she was successfully removed from mech-..
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anical circulation support in 3 days. Finally, renal function was normalized and LVEF remarkably recovered (58%) 60 days and 100 days
after the administration of steroid therapy, respectively. We continued cardioprotective drugs and prednisone paying attention to theclinical course. Cardiac magnetic resonance imaging (CMR), which
was performed after the recovery of renal function in the late phaseof the myocarditis, showed non-patchy transmural or in mesocardium late gadolinium enhancement in the anterolateral LV wall (PanelC); 18F-fluorodeoxyglucose positron emission tomography (FDGPET) revealed abnormal uptake in both LV myocardium and kidney(Panel D). Finally, electron microscopic images of the myocardium
which was procured from the right ventricular septum on admission
revealed non-caseating epithelioid granuloma (Panel E, yellow arrow),indicating cardiac sarcoidosis. In this case, not only light-microscopy
but also CMR was not conclusive for cardiac sarcoidosis diagnosis.
This emphasizes the choice of electron microscopy or FDG-PET forsarcoidosis diagnosis.

Supplementary material
Supplementary material is available at European Heart Journal - CaseReports online.
Consent: The authors confirm that written consent for submission
and publication of this case report including images and associatedtext has been obtained from the patient in line with COPE guidance.