Purpose of Review
In this state-of-the-art review, we highlight our current understanding of diagnosis, assessment, and management of cardiac sarcoidosis (CS), focusing on recently published data and expert consensus statement guidelines.
Academic interest in cardiac sarcoidosis research has increased over the past decade along with increased clinical awareness among clinicians. In 2014, the Heart Rhythm Society published the first expert consensus statement on diagnosing and managing arrhythmias associated with CS. Cardiac magnetic resonance has emerged as a valuable tool both for diagnosing CS and predicting risk of life-threatening ventricular arrhythmias based on burden of late gadolinium enhancement. Cardiac fluorodeoxyglucose-positron emission tomography now plays a role in diagnosis, risk stratification, and assessing response to immunosuppressive therapy.
Collaborative, multidisciplinary research efforts are needed to further our understanding of this rare, complex disease. Two large multicenter prospective registries—the international Cardiac Sarcoidosis Consortium and the Canadian Cardiac Sarcoidosis Research Group—are enrolling patients to help provide insights into the natural history of the disease and current treatment strategies. Future research should focus on randomized controlled trials comparing different treatment strategies and identifying and testing novel therapeutic agents.
KeywordsCardiac sarcoidosis Cardiac magnetic resonance Positron emission tomography Implantable cardioverter defibrillator Sudden cardiac death
Compliance with Ethical Standards
Conflict of Interest
Pranav Mankad, Brian Mitchell, David Birnie, and Jordana Kron declare that they have no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
- 2.•• Birnie DH, Sauer WH, Bogun F, et al. HRS expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis. Heart Rhythm. 2014;11:1304–23 First published expert consensus statement on the diagnosis and management of arrhythmias in cardiac sarcoidosis. Includes guidelines on screening and diagnosis of cardiac sarcoidosis. CrossRefGoogle Scholar
- 7.Hulten E, Agarwal V, Cahill M, et al. Presence of late gadolinium enhancement by cardiac magnetic resonance among patients with suspected cardiac sarcoidosis is associated with adverse cardiovascular prognosis: a systematic review and meta-analysis. Circ Cardiovasc Imaging. 2016;9:1–9.CrossRefGoogle Scholar
- 8.Murtagh G, Laffin LJ, Beshai JF, et al. Prognosis of myocardial damage in sarcoidosis patients with preserved left ventricular ejection fraction: risk stratification using cardiovascular magnetic resonance. Circ Cardiovasc Imaging. 2016. https://doi.org/10.1161/CIRCIMAGING.115.003738.
- 9.Coleman GC, Shaw PW, Balfour PC, Gonzalez JA, Kramer CM, Patel AR, et al. Prognostic value of myocardial scarring on CMR in patients with cardiac sarcoidosis: a systematic review and meta-analysis. JACC Cardiovasc Imaging. 10(4):411–20. https://doi.org/10.1016/j.jcmg.2016.05.009.PubMedGoogle Scholar
- 11.Kim SJ, Pak K, Kim K. Diagnostic performance of F-18 FDG PET for detection of cardiac sarcoidosis; a systematic review and meta-analysis. J Nucl Cardiol. 2019. https://doi.org/10.1007/s12350-018-01582-y.
- 15.Slart RHJA, Glaudemans AWJM, Lancellotti P, et al. A joint procedural position statement on imaging in cardiac sarcoidosis: from the Cardiovascular and Inflammation & Infection Committees of the European Association of Nuclear Medicine, the European Association of Cardiovascular Imaging, and the American Society of Nuclear Cardiology. Eur Heart J Cardiovasc Imaging. 2017;18:1073–89.CrossRefGoogle Scholar
- 19.Mankad P, Syed H, Syed A et al. C-reactive protein is elevated in sarcoidosis patients with ventricular tachycardia and heart failure. Hear. Rhythm Soc. 2019 Abstr.Google Scholar
- 24.Kron J, Chicos A, Bogun F et al (2017) Treatment of cardiac sarcoidosis with steroids and steroid-sparing immunosuppressants: findings from the Cardiac Sarcoidosis Consortium. Hear. Rhythm Soc. 2017 Abstr.Google Scholar
- 28.Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018;15:e73–e189.CrossRefGoogle Scholar
- 30.Muser D, Santangeli P, Pathak RK, et al. Long-term outcomes of catheter ablation of ventricular tachycardia in patients with cardiac sarcoidosis. Circ Arrhythm Electrophysiol. 2016;9:1–13.Google Scholar
- 31.• Yalagudri S, Zin Thu N, Devidutta S, Saggu D, Thachil A, Chennapragada S, et al. Tailored approach for management of ventricular tachycardia in cardiac sarcoidosis. J Cardiovasc Electrophysiol. 2017;28:893–902 Characterization of underlying disease in cardiac sarcoidosis via PET-CT and tailoring management of VT accordingly can help improve clinical outcomes. CrossRefGoogle Scholar
- 32.Kron J, Sauer W, Schuller J, et al. Efficacy and safety of implantable cardiac defibrillators for treatment of ventricular arrhythmias in patients with cardiac sarcoidosis. EP Eur. 2013;15:347–54.Google Scholar
- 35.• Nordenswan HK, Lehtonen J, Ekström K, et al. Outcome of cardiac sarcoidosis presenting with high-grade atrioventricular block. Circ Arrhythm Electrophysiol. 2018;11:e006145 Findings from this study provide further support for the Heart Rhythm Expert Consensus recommendation to implant intracardiac cardioverter defibrillator for cardiac sarcoidosis patient when permanent pacing is needed. CrossRefGoogle Scholar
- 36.Kusumoto FM, Schoenfeld MH, Barrett C, et al. 2018 ACC/AHA/HRS guideline on the evaluation and management of patients with bradycardia and cardiac conduction delay. Circulation. 2019. https://doi.org/10.1161/cir.0000000000000628.
- 38.Zipse M, Tzou W, Schuller J et al (2019) Electrophysiologic testing for diagnostic evaluation and risk stratification in patients with suspected cardiac sarcoidosis with preserved left and right ventricular systolic function. J Cardiovasc Electrophysiol In Press:Google Scholar