Clinical outcomes following hospitalization for COVID-19 in patients with cardiac sarcoidosis in the United States: a propensity-matched analysis from national inpatient sample database from April 2020 to December 2021.

Background: The highly arrhythmogenic nature of cardiac sarcoidosis (CS) leads to high morbidity and mortality, the rates of which may be higher in COVID-19 patients. This study aimed to evaluate the outcomes of CS patients admitted to hospitals with COVID-19. Methods: The study utilised the 2020-2021 National Inpatient Sample database, examining primary COVID-19 cases in adults aged older than or equal to 18 years. Those with CS were identified using ICD-10 code "D86.85" and compared with and without propensity matching (1:10) to those without CS for baseline characteristics and primary outcomes of acute kidney injury (AKI), use of mechanical ventilation, cardiac arrest and mortality. Results: In total, 2 543 912 COVID-19 cases were identified. Before propensity matching, CS patients were more likely to be younger (58.0 vs. 64.0 years, P<0.01), male (64.0% vs. 52.6%, P=0.011), of Black ethnicity (60.0% vs. 15.9%, P<0.01), exhibit higher Charlson Comorbidity Index (CCI) scores (3.00 vs. 1.00, P<0.01) and had a higher incidence of in-hospital cardiac arrest (aOR 2.649, 95% CI 1.366-5.134, P=0.004). After propensity matching (CS, N=95; non-CS, N=875), those with CS were at a statistically significant reduced risk of AKI (aOR 0.484, P=0.01); however, the outcomes of death, cardiac arrest, mechanical ventilation, length of stay (LOS) and healthcare costs did not reach significance. Conclusion: In a propensity-matched cohort admitted with COVID-19, CS patients had a reduced risk of AKI, but comparable LOS, rates of cardiac arrest, mechanical ventilator use, and mortality. Future research is warranted to develop evidence-based guidelines for managing COVID-19 in patients with CS.

Discordant myocardial perfusion defects and coronary artery disease - a multimodality imaging journey in the natural history of cardiac sarcoidosis.

Cardiac sarcoidosis is a rare form of systemic sarcoidosis characterized by formation of non-caseating granulomas in the myocardium, leading to heterogeneous manifestations, including conduction disturbances, arrhythmias, and heart failure. Besides myocardial fibrosis and structural myocardial alterations, cardiac sarcoidosis can impact the epicardial and microvascular circulation, causing ischemia and regional microvascular dysfunction. We present a case of cardiac sarcoidosis with atypical initial presentation but with evidence of disease in myocardial perfusion imaging, which was overlooked since it did not correspond to bystander coronary artery disease. Multimodality imaging facilitated diagnosis and offered insights into the regional microcirculation impairment caused by sarcoidosis-induced inflammation.

Current Practices and Emerging Therapies to Optimize Heart Failure Management in Cardiac Sarcoidosis: A Systematic Review.

Cardiac sarcoidosis (CS) is a distinctive manifestation of sarcoidosis, a multisystemic inflammatory disorder that is characterized by non-necrotizing granulomas. CS can lead to arrhythmias, heart failure (HF), and sudden cardiac death. The diagnosis of CS involves imaging in the form of a two-dimensional echocardiogram, cardiac magnetic resonance imaging (MRI), an 18-fluoro-deoxyglucose positron emission tomography (FDG-PET) scan, and an endomyocardial biopsy. Treatment of CS entails corticosteroids, immunosuppressive agents, monoclonal antibodies, and, in advanced cases, heart transplantation (HTx). This systematic review follows Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 guidelines, focusing on HF in sarcoidosis patients. Eligibility criteria include recent (2019-2024) research papers on sarcoidosis-induced heart failure, excluding other causes. The databases searched were PubMed, Google Scholar, and ScienceDirect. From 36,755 initial articles, 2,060 remained after filtering, and 17 were selected for quality assessment. Based on quality assessment, 11 studies were included in the final review. In CS, a variety of treatment strategies can be implemented. Corticosteroids are the first-line therapeutic options, and in the majority of cases, they are very successful in controlling the disease progression. Immunosuppressive agents like methotrexate and azathioprine are used to avoid long-term steroid use. Both corticosteroids and immunosuppressives act by reducing inflammation and preventing myocardial scarring. Biological agents like infliximab and adalimumab prevent disease progression by targeting specific inflammatory pathways and are used in refractory cases. Regular HF management drugs like angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blockers (ARBs), sodium-glucose transport protein 2 (SGLT2) inhibitors, beta-blockers, and diuretics help in optimizing cardiac function. In severe cases, a left ventricular assist device (LVAD) may be required. The ultimate treatment for end-stage CS is HTx, which has to be supplemented with a strong, individualized regimen of glucocorticoids and immunosuppressives to avoid graft rejection and to control sarcoidosis. Due to a lack of standard protocols for management and limited knowledge about CS, the ideal treatment of HF is still a matter of debate. Hence, further research and clinical trials need to be performed to optimize patient outcomes.

Specific locations of myocardial inflammation and fibrosis are associated with higher risk of events in cardiac sarcoidosis.

BACKGROUND: 18F-FDG-PET/MR can identify inflammation and fibrosis, high-risk features in cardiac sarcoidosis. OBJECTIVE: To evaluate whether the involvement of certain myocardial segments is associated with higher risk compared to others. METHODS: 124 patients with suspected clinical sarcoidosis underwent 18F-FDG-PET/MR. Late gadolinium enhancement (LGE) and focal 18F-FDG uptake were evaluated globally and in the 16 myocardial segments. Presence of LGE was defined when the percentage of LGE exceeded 5.7% globally (relative to myocardial volume) and in each myocardial segment. Patients were followed up for 5.5 years. Events were defined as ventricular arrhythmia (VA, including sustained ventricular tachycardia, ventricular fibrillation, and appropriate ICD discharge), heart failure hospitalization, or all-cause death. RESULTS: Mean age was 57.1±8.9 years, and 39.5% were female; 22 patients (17.6%) had an event at follow-up, and 9 (7.2%) presented VA. LGE and 18F-FDG uptake were more frequent in patients with than without events (36.4% vs 7.8%, p=0.001). Presence of LGE or 18F-FDG in the basal anterior segment were independent predictors for events after adjustment by left ventricular ejection fraction and relative enhanced volume (LGE: OR 10.5[1.2-92.4]; p=0.034;18F-FDG: OR 5.5[1.1-27.5], p=0.038). LGE presence in basal to mid anterior, mid anteroseptal, and basal to mid inferoseptal segments was an independent predictor for VA. Presence of 18F-FDG in basal to mid anterior, mid inferoseptal and mid inferior segments was an independent predictor for VA. CONCLUSION: Involvement of specific myocardial segments, particularly basal to mid anterior and mid septal segments, is associated with higher rates of events in patients with suspected cardiac sarcoidosis.

Rates and predictors for sustained ventricular tachycardia in patients with cardiac sarcoidosis and AV block as first cardiac presentation: Implications for device implantation.

BACKGROUND: Atrioventricular block (AVB) is a frequent initial presentation of cardiac sarcoidosis (CS), but dangerous ventricular arrhythmias (VA) can occur. Despite the scarcity of data, guidelines recommend implantable cardioverter-defibrillator (ICD) rather than a pacemaker implantation whenever a device is needed. OBJECTIVE: In this study, we aimed to establish predictors for sustained VA in patients with CS presenting with pacing indication because of an AVB. METHODS: We prospectively enrolled 112 patients with CS. Excluding those with VA, 82 patients remained and were divided into 2 groups: 34 individuals with AVB as initial presentation and 48 with other symptoms as first presentation (OSF). Both groups were compared for clinical characteristics, rates of VA, left ventricular assist device (LVAD) implantation, heart transplantation, and mortality. RESULTS: During follow-up, VA was detected in 50% in the AVB and 10.4% in the OSF group (P = .001). Death, LVAD implantation, and heart transplantation occurred in 11.8% in AVB group vs 10.4% in the OSF group (P = .847). Late gadolinium enhancement (LGE) was equally observed in both groups: 70% vs 70.5% (P = .966), whereas more patients in the AVB group exhibited abnormal positron emission tomography (PET) uptake: 86.2% vs 54.3% (P = .007). In multivariate analysis, AVB (hazard ratio [HR], 25.15), right ventricular (RV) LGE in cardiovascular magnetic resonance (CMR) (HR, 7.39) were predictors for VA occurrence, whereas the use of immunosuppressive therapy was associated with less VA (HR, 4.3). CONCLUSIONS: Patients with CS presenting with AVB have a high risk of sustained VA. Although immunosuppressive drugs may reduce the occurrence of VA, ICD implantation is reasonable, especially in case of RV LGE.

Isolated cardiac sarcoidosis: A clinical challenge.

Sarcoidosis is an inflammatory disease characterised by non-caseating granulomas of unclear aetiology. Isolated cardiac sarcoidosis (ICS) is rare and occurs when there is granulomatous infiltration of myocardial tissue without evidence of extracardiac sarcoidosis. The heterogeneity in clinical manifestations often presents a diagnostic challenge which leads to delays in treatment initiation. Our case highlights the often quiescent presentation of ICS, the importance of early treatment and the diagnostic challenges that contribute to its underdiagnosis.

Medical Therapy and Clinical Outcomes in Cardiac Sarcoidosis Patients With Systolic Heart Failure.

BACKGROUND: Cardiac sarcoidosis (CS) may result in systolic heart failure (heart failure with reduced ejection fraction [HFrEF]), but its response to guideline-directed medical therapy (GDMT) remains uncertain. METHODS AND RESULTS: We investigated 881 patients evaluated for CS to identify those with diagnosed CS, left ventricular ejection fraction (LVEF) ≤40% at diagnosis, and follow-up echocardiogram within 11-24 months. Demographics, LVEF, GDMT as quantified by Kansas City Medical Optimization (KCMO) score, and immunosuppressive treatment were recorded. The primary outcome was a composite of event-free survival (unplanned heart failure hospitalization, left ventricular assist device [LVAD]/heart transplant, or death). Seventy-nine (9%) CS patients met the inclusion criteria (35% female, median age 57 years, mean LVEF 30.9%, median New York Heart Association class II [46%], mean number of GDMT agents 1.7, and mean KCMO score 31.8). Most (87%) were treated with immunosuppressive treatment. At follow-up (median 16 months), the mean number of GDMT agents increased to 2.2 (P=0.02), and the mean KCMO score to 70.1 (P<0.001). Mean LVEF improved to 39.9% (excluding LVAD/transplant; P<0.001) and the change in LVEF was correlated with follow-up KCMO score (P<0.001). The primary outcome occurred in 13 (16%) patients and differed by KCMO score (log-rank P<0.001), but not by immunosuppressive treatment (log-rank P=0.36). CONCLUSIONS: GDMT optimization is associated with better cardiac remodeling and clinical outcomes in CS patients with HFrEF.

Contemporary Diagnostics of Cardiac Sarcoidosis: The Importance of Multimodality Imaging.

Sarcoidosis is an inflammatory condition that can affect multiple organ systems and is characterized by the formation of non-caseating granulomas in various organs, including the heart. Due to suboptimal diagnostic rates, the true prevalence and incidence of cardiac sarcoidosis (CS) remain to be determined. In patients with suspected CS, an initial examination should include 12-lead ECG or ambulatory ECG monitoring, and echocardiography with the estimation of LV, RV function, and strain rate. In patients with confirmed extracardiac sarcoidosis and with high clinical suspicion for CS, sophisticated imaging modalities, including cardiac MRI and PET, are indicated. Typical inflammation patterns and myocardial scarring should pose a high suspicion for CS. In patients without diagnosed extracardiac sarcoidosis and high clinical suspicion, although with low diagnostic probability, an endomyocardial biopsy should be considered to establish the diagnosis of definite isolated cardiac sarcoidosis. Timely diagnosis enables the initiation of therapy and close monitoring of adverse cardiac events that can be life-threatening, including sudden cardiac death, ventricular tachycardia, high-degree AV block, and heart failure. Implementing biomarkers in correlation to cardiac imaging can determine the disease's severity and progression but can also be helpful in following the treatment response. The formation of larger global registries can be helpful in the identification of independent predictors of adverse clinical events and the development of specific diagnostic algorithms to reduce the overall risk of this serious condition.

Arrhythmic prognosis according to left ventricular systolic dysfunction severity in cardiac sarcoidosis.

BACKGROUND: Current guidelines present varying classes of recommendations for implantable cardioverter-defibrillator (ICD) utilization in patients with cardiac sarcoidosis (CS) and left ventricular ejection fraction (LVEF) <50%. OBJECTIVE: The purpose of this study was to investigate the ventricular arrhythmia risk in CS patients with ICDs and varying degrees of left ventricular systolic dysfunction. METHODS: The study included CS patients with an ICD and LVEF <50% at index evaluation. The primary outcome was survival free of sustained ventricular tachycardia (VT)/ventricular fibrillation (VF) after ICD implantation and was assessed comparatively for LVEF ≤35% vs 36%-49% and for primary vs secondary prevention ICD indication. RESULTS: The study included 61 patients (median age 57 years; 61% male) with LVEF 36%-49% (n = 23) or LVEF ≤35% (n = 38). An ICD was implanted for secondary prevention in 24% and 44% of the LVEF ≤35% and 36%-49% groups, respectively (P = .11). The primary outcome did not differ between the 2 groups in univariable analysis (LVEF ≤35% vs 36%-49%: hazard ratio [HR] 0.85; 95% confidence interval [CI] 0.39-1.82; P = .67). In multivariable analysis, secondary prevention ICD indication was the only significant predictor of incident sustained VT/VF (HR 2.86; 95% CI 1.23-6.67; P = .015). Mean sustained VT/VF event burden was higher in the secondary compared with the primary prevention ICD patients (0.47 vs 0.11 events per patient-year; P = .005) but did not differ significantly between LVEF ≤35% and 36%-49% patients. CONCLUSION: CS patients with ICD indications and LVEF 36%-49% carry similarly high arrhythmic risk as those with LVEF ≤35%. Patients with secondary prevention ICDs have the highest overall risk.

Outcomes of definite vs probable/presumed cardiac sarcoidosis: a systematic review and meta-analysis.

BACKGROUND: Diagnosing cardiac sarcoidosis (CS), which can be associated with arrhythmias and heart failure, remains challenging despite multiple advances over time. The 2014 Heart Rhythm Society (HRS) consensus statement recommends an endomyocardial biopsy (EMB) to establish a definite diagnosis of CS. In the absence of a positive EMB, a diagnosis of probable or presumed CS is made on the basis of clinical and imaging criteria. OBJECTIVE: To investigate whether there is any difference in outcomes between definite vs probable/presumed CS. METHODS: PubMed/MEDLINE, Embase, and the Cochrane Library databases were searched for relevant studies published after 2014. Risk ratios (RR) with 95% confidence intervals (CI) were calculated using the random effects model and presented in forest plots. RESULTS: 6 studies involving 2,204 patients were identified. The cohort had a mean age of 56.8 years (SD: ±13.6 years). The median duration of follow-up was 40.5 months. No statistically significant difference was observed between definite and probable/presumed CS for reduced risk of the composite endpoint (RR: 1.80, 95% CI: 0.93 to 3.49), and all-cause death (RR: 1.01, 95% CI: 0.48 to 2.10). CONCLUSION: This meta-analysis demonstrated the equivalence of clinical course and prognosis between definite and probable/presumed CS. This highlights the importance of a multi-disciplinary approach to CS care and emphasizes that histological confirmation should not be a prerequisite to diagnose or manage this condition.

Cardiac sarcoidosis treated with nonsteroidal immunosuppressive therapy.

Background: Nonsteroidal immunosuppressive therapy is a potential therapeutic strategy for cardiac sarcoidosis. However, it is not recommended as an established treatment option. This study aimed to demonstrate the clinical outcomes of patients with cardiac sarcoidosis using nonsteroidal immunosuppressants through the ILLUstration of the Management and PrognosIs of JapaNese PATiEnts with Cardiac Sarcoidosis multicenter retrospective registry. Methods: From a cohort of 512 patients, 426 who received corticosteroid therapy and 26 who received other immunosuppressive therapy were included for analysis. Clinical outcomes included all-cause death, fatal ventricular arrhythmic events (FVAE), and worsening heart failure with hospitalization. Results: Nonsteroidal immunosuppressants were used for retained fluorodeoxyglucose uptake in the heart (n = 14), corticosteroid side effects (n = 7), ventricular arrhythmia (n = 4), complete atrioventricular block (n = 2), worsened extracardiac sarcoidosis (n = 2), and other reasons (n = 2). They comprised of methotrexate (n = 20), cyclosporine (n = 2), cyclophosphamide (n = 2), and azathioprine (n = 3). After the addition of a nonsteroidal immunosuppressant, corticosteroids were reduced in 14 of 26 patients (5 [5-17] mg), although no patient discontinued corticosteroids. Of the 14 patients, decreased fluorodeoxyglucose uptake was observed in seven at follow-up. Clinical outcomes were observed in 11 patients (42.3 %). Detected events included all-cause death in five patients (19.2 %), FVAE in four (15.4 %), and worsening heart failure with hospitalization in five (19.2 %), with some overlap. Conclusions: Nonsteroidal immunosuppressive therapy may be a possible treatment option for patients who are not stabilized with corticosteroids alone or develop corticosteroid side effects.

[Arrhythmias in sarcoidosis]. / Arrhythmien bei Sarkoidose.

Cardiac sarcoidosis (CS) is difficult to diagnose and often requires a careful evaluation of numerous diagnostic findings. Typical features at initial presentation are a high-grade atrioventricular (AV) block and ventricular tachycardias that cannot be explained by other common entities, especially in younger patients. CS is frequently misdiagnosed and inappropriately treated, which may have deleterious consequences for the patients. In this review article, we focus on special features of the arrhythmias typical of sarcoidosis and also discuss the underlying substrate and the approach in special situations. Furthermore, we provide recommendations from our daily clinical experience, discuss open questions, and explain the need for research.

Risk Stratification in Cardiac Sarcoidosis With Cardiac Positron Emission Tomography: A Systematic Review and Meta-Analysis.

BACKGROUND: Although positron emission tomography (PET) imaging is well established for its diagnostic role in cardiac sarcoidosis, less is known about the prognostic value of PET and its use in risk stratification for major adverse cardiac events (MACE). OBJECTIVES: The goal of this study was to perform a systematic review and meta-analysis looking at the prognostic value of PET imaging in patients with cardiac sarcoidosis. METHODS: Study investigators systematically searched EMBASE (Excerpta Medica dataBASE), MEDLINE, PubMed, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, CINAHL (Cumulative Index to Nursing and Allied Health Literature), ClinicalTrials.gov, and the European Union Clinical Trial Registry for cardiac sarcoidosis and PET imaging. The primary outcome of interest was MACE. RESULTS: The search revealed 3,010 records, of which 55 studies were included. This represented 5,250 patients. Factors associated with MACE included the following: the combination of abnormal fluorodeoxyglucose (FDG) uptake and perfusion defect, which had an OR of 2.86 (95% CI: 1.74-4.71; P < 0.0001); abnormal perfusion or FDG uptake, which had an OR of 2.69 (95% CI: 1.67-4.33); abnormal FDG uptake, which had an OR of 2.61 (95% CI: 1.51-4.50); focal abnormal right ventricular uptake, which had an OR of 6.27 (95% CI: 3.19-12.32; P < 0.00001); and a lack of response to immunosuppression on serial PET, which had an OR of 8.43 (95% CI: 3.25-21.85; P < 0.0001). A QUIPS (Quality in Prognostic Studies) tool analysis found a low to moderate risk of bias, particularly given the small sample sizes in the individual studies. CONCLUSIONS: Multiple cardiac PET parameters provide risk stratification value in cardiac sarcoidosis. Focal right ventricular uptake and a lack of response to immunosuppressive therapy on serial PET imaging were particularly predictive of MACE.

30-Year Trends in the Incidence, Characteristics, and Outcome of Cardiac Sarcoidosis in a Nationwide Cohort.

Background: Cardiac sarcoidosis (CS) is a rare but potentially fatal inflammatory cardiomyopathy. Objectives: The authors studied temporal changes in the incidence, characteristics, and outcome of CS. Methods: A retrospective analysis was made of a 30-year nationwide cohort of CS. Results: The cohort comprised 511 patients with a median age of 52 years and female preponderance (69%). Altogether 77, 166, and 268 cases of CS were diagnosed in years 1988 to 2009, 2010 to 2014, and 2015 to 2019, respectively; the 5-year count of 2015 to 2019 was 134-fold the count of 1990 to 1994 (268/2) and 18-fold the count of 2000 to 2004 (268/15). Prior to 2010, compared with the later periods, CS presented more often with ventricular tachycardia/fibrillation (prevalence 36% vs 19% in 2010-2014 and 11% in 2015-2019, P < 0.001), left ventricular ejection fraction <50% (49%, 35%, and 31%; P = 0.010), and elevation of natriuretic peptides (87%, 57%, and 49%; P < 0.001). On magnetic resonance imaging, late gadolinium enhancement involved a median of 15% (IQR: 11%-22%) of left ventricular mass in studies of 1988 to 2009 (n = 16), 15% (IQR: 9%-22%) in studies of 2010 to 2014 (n = 87), and 11% (IQR: 5%-19%) in studies of 2015 to 2019 (n = 150) (P = 0.031). The respective 5-year incidences of the composite of death, heart transplantation, left ventricular-assisted device implantation, or ventricular tachyarrhythmia were 40% (95% CI: 29%-51%), 32% (95% CI: 25%-39%), and 23% (95% CI: 16%-30%) (P = 0.002). The prognostic trend disappeared after adjustment for differences in the presenting phenotype. Conclusions: Diagnoses of incident CS have increased exponentially in Finland. Concurrently, the phenotype has turned milder and prognosis better, suggesting detection of CS at an earlier stage of its course.

Association Between Right Bundle Branch Block and Ventricular Arrhythmia in Patients With Cardiac Sarcoidosis.

Background: Ventricular arrhythmia (VA) is a life-threatening condition associated with cardiac sarcoidosis (CS). Right bundle branch block (RBBB) is a common conduction disorder in CS; however, its association with VA remains unknown. Objectives: This study aimed to investigate the relationship between RBBB and VA in patients with CS. Methods: This was a post hoc analysis of ILLUMINATE-CS (Illustration of the Management and Prognosis of Japanese Patients with Cardiac Sarcoidosis), a multicenter, retrospective, and observational study that evaluated the clinical characteristics and prognosis of CS. Eligible patients were divided into two groups based on the presence or absence of RBBB at the time of diagnosis. The primary outcome was serious ventricular arrhythmia events (SVAEs), defined as a combination of sudden cardiac death and documented ventricular fibrillation, sustained ventricular tachycardia, or appropriate implantable cardioverter-defibrillator therapy. Results: Overall, 312 patients were studied, with 155 (49.7%) patients presenting with RBBB (RBBB group). Patients in the RBBB group had a higher prevalence of basal interventricular septum (IVS) thinning and prominent late gadolinium enhancement in the basal IVS on cardiac magnetic resonance imaging than those in the non-RBBB group. During a median follow-up of 3.0 years (IQR: 1.6-6.0 years), 66 patients experienced SVAE. In multivariable Cox regression analysis, the RBBB group was independently associated with a higher incidence of SVAEs (HR: 1.93 [95% CI: 1.14-3.28]; P = 0.015). Conclusions: In patients with CS, RBBB was an independent predictor of SVAEs, which might reflect the specific scar distribution that is predominant in the IVS.

Cardiac sarcoidosis presenting as multiple right intra-atrial masses mimicking cardiac tumor.

BACKGROUND: Cardiac sarcoidosis though in itself, a rare entity, very rarely presents primarily with conduction abnormalities as the primary manifestation in the spectrum of presentations accounted by this chronic granulomatous systemic disease. Sarcoidosis presenting as intra-atrial masses is virtually unheard of. CASE: A middle aged female presented with progressive conduction system disease was found to have right atrial masses of unclear etiologic on relevant imaging. Over the course of 3 months she underwent a dual-chamber ICD implant for her eventual complete heart block and a surgical resection following an inconclusive biopsy of the right atrial free wall mass. She was then diagnosed with cardiac sarcoidosis and started on immunosupressants almost instantaneously as a part of her treatment. CONCLUSION: This is an entirely new and unreported presentation of cardiac sarcoidosis as an intra-atrial mass. Through this case we bring light to cardiac sarcoidosis as a potential differential for intra-cardiac masses and how with available data do we go about treating it.

Cardiac sarcoidosis manifesting with atrioventricular block and intracardiac masses: case report and literature review.

Background: Cardiac sarcoidosis (CS) typically manifests with atrioventricular block (AVB), ventricular arrhythmias, or heart failure. Intracardiac masses due to CS are rare, and there is both a paucity of evidence and guidelines of how manage them. Case summary: We describe a 45-year-old woman who presented with palpitations and dyspnoea on exertion found to have second-degree AVB. Further work-up noted two right atrial masses that, following excision and pathology, were identified as CS. Within several months of immunosuppressive treatment, imaging and device reports demonstrated mass resolution without arrhythmia recurrence. Discussion: Intracardiac masses are a rare manifestation of CS. Immunosuppressive therapy remains the mainstay of treatment, with consideration of mass resection for diagnostic purposes.