J-STAGE Advance Publication released online October 9, 2019 Mailing address: Scientific Committee of the Japanese Circulation Society, 18F Imperial Hotel Tower, 1-1-1 Uchisaiwai-cho, Chiyoda-ku, Tokyo 100-0011, Japan. E-mail: meeting@j-circ.or.jp This English document is a digest version of JCS 2016 Guideline on Diagnosis and Treatment of Cardiac Sarcoidosis reported at the Japanese Circulation Society Joint Working Groups perfomed in 2016. (Website: http://www.j-circ.or.jp/guideline/pdf/ JCS2016_terasaki_d.pdf). Refer to Appendix 1 for the detailes of members. Joint Working Groups: The Japanese Circulation Society, Japanese College of Cardiology, The Japanese Heart Failure Society, Japan Society of Sarcoidosis and Other Granulomatous Disorders, The Japanese Society of Nuclear Cardiology, Japanese Heart Rhythm Society, The Research Group of Idiopathic Cardiomyopathy, Health, Labour and Welfare Sciences Research Grants, Research on Rare and Intractable Diseases. ISSN-1346-9843 All rights are reserved to the Japanese Circulation Society. For permissions, please e-mail: cj@j-circ.or.jp JCS 2016 Guideline on Diagnosis and Treatment of Cardiac Sarcoidosis ― Digest Version ―

JCS 2016 Guideline on Diagnosis and Treatment of Cardiac Sarcoidosis - Digest Version

Clinically evident sarcoidosis involving the heart has been noted in at least 2 to 7% of patients with sarcoidosis, but occult involvement is much higher (> 20%). Cardiac sarcoidosis is often not recognized antemortem, as sudden death may be the presenting feature. Cardiac involvement may occur at any point during the course of sarcoidosis and may occur in the absence of pulmonary or systemic involvement. Sarcoidosis can involve any part of the heart, with protean manifestations. Prognosis of cardiac sarcoidosis is related to extent and site(s) of involvement. Most deaths due to cardiac sarcoidosis are due to arrhythmias or conduction defects, but granulomatous infiltration of the myocardium may be lethal. The definitive diagnosis of isolated cardiac sarcoidosis is difficult. The yield of endomyocardial biopsies is low; treatment of cardiac sarcoidosis is often warranted even in the absence of histologic proof. Radionuclide scans are integral to the diagnosis. Currently, 18F-fluorodeoxyglucose positron emission tomography/computed tomography and gadolinium-enhanced magnetic resonance imaging scans are the key imaging modalities to diagnose cardiac sarcoidosis. The prognosis of cardiac sarcoidosis is variable, but mortality rates of untreated cardiac sarcoidosis are high. Although randomized therapeutic trials have not been done, corticosteroids (alone or combined with additional immunosuppressive medications) remain the mainstay of treatment. Because of the potential for sudden cardiac death, implantable cardioverter-defibrillators should be placed in any patient with cardiac sarcoidosis and serious ventricular arrhythmias or heart block, and should be considered for cardiomyopathy. Cardiac transplantation is a viable option for patients with end-stage cardiac sarcoidosis refractory to medical therapy.

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Cardiac Involvement in Sarcoidosis: Evolving Concepts in Diagnosis and Treatment

Spectrum of Restrictive and Infiltrative Cardiomyopathies: Part 2 of a 2-Part Series

Objectives The aim of this study was to identify differences in survival on the basis of type of heart disease while awaiting orthotopic heart transplantation (OHT) Background Patients with restrictive cardiomyopathy (RCM), congenital heart disease (CHD), or hypertrophic cardiomyopathy (HCM) may be at a disadvantage while awaiting OHT because they often are poor candidates for mechanical circulatory support and/or inotropes Methods The study included all adults in the Scientific Registry of Transplant Recipients database awaiting OHT from 2004 to 2014, and outcomes were evaluated on the basis of type of heart disease The primary endpoint was time to all-cause mortality, censored at last patient follow-up and time of transplantation Multivariate Cox proportional hazards modeling was performed to evaluate survival by type of cardiomyopathy Results There were 14,447 patients with DCM, 823 with RCM, 11,799 with ischemic cardiomyopathy (ICM), 602 with HCM, 964 with CHD, 584 with valvular disease, and 1,528 in the “other” category (including 1,216 for retransplantation) During median follow-up of 37 months, 4,943 patients died (1,253 women, 3,690 men) After adjusting for possible confounding variables including age, renal function, inotropes, mechanical ventilation, and mechanical circulatory support, the adjusted hazard ratios by diagnoses relative to DCM were 170 for RCM (95% confidence interval [CI]: 143 to 202), 110 for ICM (95% CI: 103 to 118), 123 for HCM (95% CI: 098 to 154), 130 for valvular disease (95% CI: 107 to 157), 137 for CHD (95% CI: 117 to 161), and 151 for “other” diagnoses (95% CI: 134 to 169) Sex was a significant modifier of mortality for ICM, RCM, and “other” diagnoses (p  Conclusions In the United States, patients with RCM, CHD, or prior heart transplantation had a higher risk for death while awaiting OHT than patients with DCM, ICM, HCM, or valvular heart disease

Does Survival on the Heart Transplant Waiting List Depend on the Underlying Heart Disease

Cardiac sarcoidosis is a rare but potentially fatal disorder with a nonspecific spectrum of clinical manifestations, including conduction disorders, congestive heart failure, ventricular arrhythmias, and sudden cardiac death. Although early treatment to improve morbidity and mortality is desirable, sensitive and accurate detection of cardiac sarcoidosis remains a challenge. Except for the histopathologic finding of noncaseating granulomas in an endomyocardial biopsy specimen, most diagnostic tests are limited and nonspecific at best. Therefore, the decision to initiate treatment is based largely on the patient's clinical symptoms and the course of the disease, rather than histologic confirmation. Successful recognition of cardiac sarcoidosis ultimately requires rigorous collaboration among a clinician, radiologist, and pathologist. Advanced imaging modalities, such as cardiac magnetic resonance imaging and positron emission tomography with fluorodeoxyglucose, have become increasingly useful in facilitating diagnosis and therapeutic monitoring, although limited prospective studies exist. This article describes the clinical parameters and pathologic findings of cardiac sarcoidosis and the advanced imaging features and differential diagnostic challenges that must be considered for a successful diagnostic approach. In addition, to improve the understanding of abnormalities detected with different imaging modalities, we suggest a unified terminology in describing radiologic findings related to cardiac sarcoidosis.

Cardiac Sarcoidosis: The Challenge of Radiologic-Pathologic Correlation: From the Radiologic Pathology Archives

Background Cardiac sarcoidosis with end-stage heart failure has a poor prognosis without transplantation The rates of sarcoid recurrence and rejection are not well established after heart transplantation Methods A total of 19 heart transplant recipients with sarcoid of the explanted heart were compared with a contemporaneous control group of 1,050 heart transplant recipients without cardiac sarcoidosis Assessed outcomes included 1st-year freedom from any treated rejection, 5-year actuarial survival, 5-year freedom from cardiac allograft vasculopathy (CAV), 5-year freedom from nonfatal major adverse cardiac events (NF-MACE), and recurrence of sarcoid in the allograft or other organs Patients with sarcoidosis were maintained on low-dose corticosteroids after transplantation Results There were no significant differences between the sarcoid and control groups in 1st-year freedom from any treated rejection (79% and 90%), 5-year posttransplantation survival (79% and 83%), 5-year freedom from CAV (68% and 78%), and 5-year freedom from NF-MACE (90% and 88%) Causes of death (n = 5) in the sarcoid group were coccidioidomycosis, pneumonia, rejection, hemorrhage, and CAV No patient had recurrence of sarcoidosis in the cardiac allograft Three of 19 patients (16%) experienced recurrence of extracardiac sarcoid, with no mortality Conclusions Patients with cardiac sarcoidosis undergoing heart transplantation have acceptable long-term outcomes without evidence of recurrence of sarcoidosis in the allograft when maintained on low-dose corticosteroids Progression of extracardiac sarcoid was uncommon, possibly related to immunosuppression In patients with cardiac sarcoidosis, heart transplantation is a viable treatment modality

D.P. Perkel

Papers

Heart transplantation for end-stage heart failure due to cardiac sarcoidosis.

Multivalvular abscesses in a patient with corynebactererium striatum bacteremia and undiagnosed thymoma