IMAGES THAT TEACH Isolated right ventricular cardiac sarcoidosis demonstrated by 18FDG positron emission tomography Miguel Hernandez Pampaloni, MD, PhD,a Babek Nazar, MD,b and Elias Botvinick, MDb a b
Department of Radiology & Biomedical Imaging, UCSF, San Francisco, CA Division of Cardiology, Department of Medicine, UCSF, San Francisco, CA
Received May 2, 2013; accepted Dec 13, 2013 doi:10.1007/s12350-013-9848-2
The patient is a 40-year-old male who initially presented with shortness of breath and dyspnea on exertion in March of 2011. Initial evaluation demonstrated a right bundle branch block and QRS fragmentation on electrocardiogram (ECG) and hilar lymphadenopathy on chest x-ray. Transthoracic echocardiogram demonstrated normal left ventricular systolic function, and no other abnormalities. A treadmill stress test demonstrated excellent exercise capacity with no ECG evidence of ischemia. Bronchoscopic hilar lymph node biopsy showed non-caseating granulomas, consistent with sarcoidosis. Out of concern for cardiac involvement, a low carbohydrate diet cardiac PET scan was ordered. 82Rb resting scan demonstrated normal left ventricular perfusion and systolic function. 18FDG scan demonstrated 18FDG uptake in the right ventricle and hilar lymph nodes, but not in the left ventricle (Figures 1, 2A, C). Gadolinium-enhanced cardiac MRI showed dyskinesis and delayed gadolinium enhancement of the right ventricular (RV) free wall, mild RV enlargement, and an RV ejection fraction of 36 % (Figure 3). A 5-day event monitor demonstrated
Reprint requests: Miguel Hernandez Pampaloni, Department of Radiology & Biomedical Imaging, UCSF, San Francisco, CA; [email protected] J Nucl Cardiol 1071-3581/$34.00 Copyright Ó 2014 American Society of Nuclear Cardiology.
premature ventricular contractions accounting for 2.8 % of beats, with ventricular bigeminy and trigeminy, but no sustained ventricular tachycardia. The patient received an implantable cardioverter defibrillator (ICD) for primary prevention of sudden cardiac death, and subsequent interrogation demonstrated 10- to 15-second-long runs on non-sustained ventricular tachycardia on an approximately daily basis. The patient was subsequently started on prednisone 40 mg daily after consultation with his pulmonologist and cardiologist. One year later, the patient underwent a repeat cardiac PET scan showing normal 82Rb left ventricular perfusion, and persistent, but decreased intensity of 18 FDG uptake in the right ventricle (Figure 2B). Given the continued 18FDG uptake suggestive of RV inflammation, methotrexate was added to the patient’s regimen. Interrogation of his ICD demonstrated improvement of his ventricular arrhythmias, and he has since done well, with no symptoms suggestive of congestive heart failure. Clinical cardiac involvement is seen in approximately 5 % of patients with sarcoidosis, but clinically silent myocardial involvement has been noted in 25 % of autopsy cases of patients with systemic sarcoidosis.1 Cardiac manifestations include conduction system abnormalities, ventricular and supraventricular arrhythmias, valvular dysfunction, and left ventricular dysfunction.2 There are no randomized clinical trials to guide the decision to initiate immunosuppressive therapy or implant an ICD, but retrospective analyses have shown a clinical improvement 3 and prolonged survival 4 with glucocorticoid therapy. Isolated RV cardiac sarcoidosis is rare, and has only been reported in case reports, mimicking arrhythmogenic right ventricular cardiomyopathy (ARVC).5,6 Our case is the first to demonstrate corroborating 18FDGPET and MRI imaging of isolated RV sarcoidosis, with
Hernandez Pampaloni et al Isolated right ventricular cardiac sarcoidosis
a beneficial clinical response to corticosteroids as evidenced by decreased ventricular arrhythmia burden and improved RV inflammation on 18FDG-PET. The right
Figure 1. Whole-body maximum intensity projection 18FFDG images showing numerous bilateral hypermetabolic (indicated by the arrows) hilar, mediastinal, paraesophageal and retrocrural lymphadenopathy, consistent with systemic sarcoidosis.
Journal of Nuclear CardiologyÒ
ventricle should be added to clinicians’ differential diagnosis for potential cardiac targets of sarcoidosis. Furthermore, corticosteroids and other immunosuppressive agents should be considered for the treatment of RV sarcoidosis, as they are in other forms of cardiac sarcoidosis.
Figure 3. Transaxial mid-ventricular magentic resonance image after the intravenous administration of gadolinium demonstrating an area of delayed enhancement at the level of the RV (white arrow), suggestive of necrosis.
Figure 2. Transaxial mid-ventricular cardiac PET images. (A) demonstrates increased FDG uptake in the region of the free wall of the right ventricle (white arrow). This increased glucose utilization rate is consistent with an inflammatory process and compatible with myocardial sarcoidosis. (SUVm 7.8). (B) represents metabolic FDG-PET study 1 year later, showing increased, but diminished when compared with (A), FDG uptake in the right ventricle (white arrow SUVm 4.6), suggestive of partial response to therapy. (C) shows the homogenous blood flow perfusion at rest within the left ventricle with N-13 ammonia PET.
Journal of Nuclear CardiologyÒ
References 1. Thomsen TK, Eriksson T. Myocardial sarcoidosis in forensic medicine. Am J Forensic Med Pathol 1999;20:52. 2. Kim JS, Judson MA, Donnino R, Gold M, Cooper LT Jr, Prystowsky EN, et al. Cardiac sarcoidosis. Am Heart J 2009;157:9. 3. Chapelon-Abric C, de Zuttere D, Duhaut P, Veyssier P, Wechsler B, Huong DL, et al. Cardiac sarcoidosis: A retrospective study of 41 cases. Medicine 2004;83:315. 4. Takada K, Ina Y, Yamamoto M, Satoh T, Morishita M. Prognosis after pacemaker implantation in cardiac sarcoidosis in Japan.
Hernandez Pampaloni et al Isolated right ventricular cardiac sarcoidosis
Clinical evaluation of corticosteroid therapy. Sarcoidosis 1994;11:113. 5. Mohsen A, Panday M, Wetherold S, Jimenez A. Cardiac sarcoidosis mimicking arrhythmogenic right ventricular dysplasia with high defibrillation threshold requiring subcutaneous shocking coil implantation. Heart Lung Circ 2012;21:46-9. 6. Ladvjanskaia GA, Basso C, Hobbelink MG, Kirkels JH, Lahpor JR, Cramer MJ, et al. Sarcoid myocarditis with ventricular tachycardia mimicking ARVD/C. J Cardiovasc Electrophysiol 2010;21:94-8.
Department of Radiology & Biomedical Imaging, UCSF, San Francisco, CA Division of Cardiology, Department of Medicine, UCSF, San Francisco, CA
Received May 2, 2013; accepted Dec 13, 2013 doi:10.1007/s12350-013-9848-2
The patient is a 40-year-old male who initially presented with shortness of breath and dyspnea on exertion in March of 2011. Initial evaluation demonstrated a right bundle branch block and QRS fragmentation on electrocardiogram (ECG) and hilar lymphadenopathy on chest x-ray. Transthoracic echocardiogram demonstrated normal left ventricular systolic function, and no other abnormalities. A treadmill stress test demonstrated excellent exercise capacity with no ECG evidence of ischemia. Bronchoscopic hilar lymph node biopsy showed non-caseating granulomas, consistent with sarcoidosis. Out of concern for cardiac involvement, a low carbohydrate diet cardiac PET scan was ordered. 82Rb resting scan demonstrated normal left ventricular perfusion and systolic function. 18FDG scan demonstrated 18FDG uptake in the right ventricle and hilar lymph nodes, but not in the left ventricle (Figures 1, 2A, C). Gadolinium-enhanced cardiac MRI showed dyskinesis and delayed gadolinium enhancement of the right ventricular (RV) free wall, mild RV enlargement, and an RV ejection fraction of 36 % (Figure 3). A 5-day event monitor demonstrated
Reprint requests: Miguel Hernandez Pampaloni, Department of Radiology & Biomedical Imaging, UCSF, San Francisco, CA; [email protected] J Nucl Cardiol 1071-3581/$34.00 Copyright Ó 2014 American Society of Nuclear Cardiology.
premature ventricular contractions accounting for 2.8 % of beats, with ventricular bigeminy and trigeminy, but no sustained ventricular tachycardia. The patient received an implantable cardioverter defibrillator (ICD) for primary prevention of sudden cardiac death, and subsequent interrogation demonstrated 10- to 15-second-long runs on non-sustained ventricular tachycardia on an approximately daily basis. The patient was subsequently started on prednisone 40 mg daily after consultation with his pulmonologist and cardiologist. One year later, the patient underwent a repeat cardiac PET scan showing normal 82Rb left ventricular perfusion, and persistent, but decreased intensity of 18 FDG uptake in the right ventricle (Figure 2B). Given the continued 18FDG uptake suggestive of RV inflammation, methotrexate was added to the patient’s regimen. Interrogation of his ICD demonstrated improvement of his ventricular arrhythmias, and he has since done well, with no symptoms suggestive of congestive heart failure. Clinical cardiac involvement is seen in approximately 5 % of patients with sarcoidosis, but clinically silent myocardial involvement has been noted in 25 % of autopsy cases of patients with systemic sarcoidosis.1 Cardiac manifestations include conduction system abnormalities, ventricular and supraventricular arrhythmias, valvular dysfunction, and left ventricular dysfunction.2 There are no randomized clinical trials to guide the decision to initiate immunosuppressive therapy or implant an ICD, but retrospective analyses have shown a clinical improvement 3 and prolonged survival 4 with glucocorticoid therapy. Isolated RV cardiac sarcoidosis is rare, and has only been reported in case reports, mimicking arrhythmogenic right ventricular cardiomyopathy (ARVC).5,6 Our case is the first to demonstrate corroborating 18FDGPET and MRI imaging of isolated RV sarcoidosis, with
Hernandez Pampaloni et al Isolated right ventricular cardiac sarcoidosis
a beneficial clinical response to corticosteroids as evidenced by decreased ventricular arrhythmia burden and improved RV inflammation on 18FDG-PET. The right
Figure 1. Whole-body maximum intensity projection 18FFDG images showing numerous bilateral hypermetabolic (indicated by the arrows) hilar, mediastinal, paraesophageal and retrocrural lymphadenopathy, consistent with systemic sarcoidosis.
Journal of Nuclear CardiologyÒ
ventricle should be added to clinicians’ differential diagnosis for potential cardiac targets of sarcoidosis. Furthermore, corticosteroids and other immunosuppressive agents should be considered for the treatment of RV sarcoidosis, as they are in other forms of cardiac sarcoidosis.
Figure 3. Transaxial mid-ventricular magentic resonance image after the intravenous administration of gadolinium demonstrating an area of delayed enhancement at the level of the RV (white arrow), suggestive of necrosis.
Figure 2. Transaxial mid-ventricular cardiac PET images. (A) demonstrates increased FDG uptake in the region of the free wall of the right ventricle (white arrow). This increased glucose utilization rate is consistent with an inflammatory process and compatible with myocardial sarcoidosis. (SUVm 7.8). (B) represents metabolic FDG-PET study 1 year later, showing increased, but diminished when compared with (A), FDG uptake in the right ventricle (white arrow SUVm 4.6), suggestive of partial response to therapy. (C) shows the homogenous blood flow perfusion at rest within the left ventricle with N-13 ammonia PET.
Journal of Nuclear CardiologyÒ
References 1. Thomsen TK, Eriksson T. Myocardial sarcoidosis in forensic medicine. Am J Forensic Med Pathol 1999;20:52. 2. Kim JS, Judson MA, Donnino R, Gold M, Cooper LT Jr, Prystowsky EN, et al. Cardiac sarcoidosis. Am Heart J 2009;157:9. 3. Chapelon-Abric C, de Zuttere D, Duhaut P, Veyssier P, Wechsler B, Huong DL, et al. Cardiac sarcoidosis: A retrospective study of 41 cases. Medicine 2004;83:315. 4. Takada K, Ina Y, Yamamoto M, Satoh T, Morishita M. Prognosis after pacemaker implantation in cardiac sarcoidosis in Japan.
Hernandez Pampaloni et al Isolated right ventricular cardiac sarcoidosis
Clinical evaluation of corticosteroid therapy. Sarcoidosis 1994;11:113. 5. Mohsen A, Panday M, Wetherold S, Jimenez A. Cardiac sarcoidosis mimicking arrhythmogenic right ventricular dysplasia with high defibrillation threshold requiring subcutaneous shocking coil implantation. Heart Lung Circ 2012;21:46-9. 6. Ladvjanskaia GA, Basso C, Hobbelink MG, Kirkels JH, Lahpor JR, Cramer MJ, et al. Sarcoid myocarditis with ventricular tachycardia mimicking ARVD/C. J Cardiovasc Electrophysiol 2010;21:94-8.
