Correspondence CMR imaging for follow up of isolated cardiac sarcoidosis with extensive biventricular involvement Johannes Schwab a,b, ⁎, Klaus Fessele a , Dirk Bastian a , Joachim H. Ficker c , Thomas Papadopoulos d , Michael Lell b , Matthias Pauschinger a a Department of Cardiology, Paracelsus Medical University Nuernberg, General Hospital Nuernberg, Germany b Institute of Radiology and Nuclear Medicine, Paracelsus Medical University Nuernberg, General Hospital Nuernberg, Germany c Department of Respiratory Medicine, Allergology and Sleep Medicine, Paracelsus Medical University Nuernberg, General Hospital Nuernberg, Germany d Institute of Pathology, Paracelsus Medical University Nuernberg, General Hospital Nuernberg, Germany article info Article history: Received 1 May 2016 Accepted 28 June 2016 Available online 9 July 2016 Cardiac sarcoidosis (CS) commonly involves the heart without clin- ically apparent extracardiac disease. Heart failure is becoming the lead- ing cause of death in CS, responsible for 73% [1]. Conduction abnormalities such as right bundle branch or atrioventricular block, ventricular and supraventricular arrhythmias, and systolic or diastolic heart failure can occur [2]. A 32-year-old male presented in 07/2014 with progressive dyspnea NYHA II–III, mild chest pain and reduced exercise tolerance. Physical ex- amination including lymph nodes, eyes and skin was unremarkable, but the electrocardiogram (ECG) showed sinus rhythm with an atrioventric- ular block I° and a protein, serum protein electrophoresis, immunoglobu- lins, QuantiFERON-TB Gold Test, α-galactosidase, neopterine, serum amyloid, borrelia antibodies were within normal range. However cardiac markers were abnormal with a slightly elevated high sensitive cTnT (0.045 ng/ml, upper reference limit b 0.014 ng/ml) and a significantly elevated Nt-proBNP (1066 pg/ml, upper reference limit b 88 pg/ml). The serum concentrations of soluble interleukin 2 receptor (697 kU/l, ref- erence limits 158–623 kU/l) and angiotensin-converting enzyme (83 U/l, reference limits 20–70 U/l) were increased as well Transthoracic echocardiography revealed the presence of a normal sized LV (LVEDD 53 mm) with significant left ventricular hypertrophy (IVSd17 mm, LVPWd 18 mm) and decreased left ventricular systolic (LV EF 50% measured according to biplane Simpson's rule) and diastolic dysfunction. These findings were in contrast to a normal echocardiogra- phy examination, which was done when he was evaluated for an epi- sode of gastroenteritis with fever in 03/2013. Cardiac catheterization revealed normal coronary anatomy and the absence of coronary disease. Endomyocardial biopsy (Fig. 1e) showed mild hypertrophy with histio- cytes in a granulomatous pattern with T-lymphocytes and a multinucle- ated giant cell but neither myocyte necrosis nor eosinophils. An extensive search for sarcoidosis including chest CT scan, bronchoscopy with central biopsy, ultrasound-guided transbronchial lymph node bi- opsy and bronchoalveolar lavage revealed no histological or cytological evidence of pulmonary sarcoidosis. An initial cardiac magnetic resonance (CMR) imaging study was performed before initiation of immunosuppressive therapy, which was added due to progressive heart failure despite conventional therapy. Immunosuppressive thera- py consisted of oral prednisone 1 mg/kg/d for 4 weeks, followed by 0.33 mg/kg/d for 5 months in combination with azathioprine 50 mg/d. After 6 months the prednisone dose was tapered off every 2 weeks by 5 mg/d until a maintenance dose of 10 mg/d was reached. The initial Cine-CMR (08/2014) showed global LV- and RV-hypokinesia, reduced LV ejection fraction = 40% and RV ejection fraction = 36%, LV- and RV-hypertrophy (LV mass = 171 g/m 2 , RV mass 49 g/m 2 )(Fig. 1g) and a small pericardial effusion. T2-weighted images showed an omni- present diffuse high intensity signal with elevated edema ratio (ER = 3.0) (Fig. 1i). Late-gadolinium enhancement (LGE) was detected in a circumferential subepicardial pattern both in the LV and the RV wall and intramural in the septum (Fig. 1h). The CMR study was repeated after 3, 6 and 10 months (Table 1). After 3 months of immunosuppres- sive therapy, there was already an improvement of LV- and RV EF with normalization of ER = 1.6, resp. marked decrease of LV- and RV mass (Fig. 1 a,b), whereas there was still unchanged circumferential subepicardial LGE in the LV and RV wall. After 10 months the LV-EF ap- peared only slightly reduced (48%) and the RV-EF was normal (RV- EF = 57%) (Fig. 1j), the ER continued to be normal (ER = 1.6) (Fig. 1l), there was no pericardial effusion and LV- and RV mass had returned to normal. On visual comparison there was a decrease of LGE mainly in the septum with persisting subepicardial circumferential LGE in the LV and RV-wall (Fig. 1k). Corresponding to the improvement in the imaging studies, there was a pronounced clinical improvement (NYHA I) and normalization of biomarkers (Nt-proBNP = 159 pg/ml and high sensitive cTnT b 0.014 ng/ml) 9 months after the start of therapy (Fig. 1c,d). Cardiac sarcoidosis (CS) and giant-cell myocarditis (GCM) are rare disorders and the patients can present with a similar presentation. International Journal of Cardiology 221 (2016) 777–779 ⁎ Corresponding author at: Department of Cardiology, Paracelsus Medical University Nuernberg, General Hospital Nuernberg, , Germany. E-mail address: Johannes.schwab@klinikum-nuernberg.de (J. Schwab). http://dx.doi.org/10.1016/j.ijcard.2016.06.295 0167-5273/© 2016 Elsevier Ireland Ltd. All rights reserved. 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