Extracardiac Sarcoidosis Research Articles

Echocardiographic evaluation of left ventricular mechanicsin sarcoidosis patients without overt heart disease: a systematic review and meta-analysis.

During the last decade, a small number of studies have used speckle tracking echocardiography (STE) to investigate sarcoidosis effect on left ventricular (LV) mechanics in patients without overt heart disease. The present systematic review and meta-analysis has been primarily designed to summarize the main findings of these studies and to examine the overall influence of sarcoidosis on LV-global longitudinal strain (GLS) and left ventricular ejection fraction (LVEF). All echocardiographic studies assessing conventional echoDoppler parameters and myocardial strain indices in patients with extracardiac sarcoidosis (ECS) vs. healthy controls, selected from PubMed and EMBASE databases, were included. The risk of bias was assessed by using the National Institutes of Health (NIH) Quality Assessment of Case-Control Studies. Continuous data (LV-GLS and LVEF) were pooled as a standardized mean difference (SMD) comparing sarcoidosis group with healthy controls. The overall SMDs of LV-GLS and LVEF were calculated using the random-effect model. The full-text of 13 studies with 785 ECS patients and 567 healthy controls were analyzed. Both average LVEF (60.5±6.6 vs 63.0±4.8%, P<0.001) and LV-GLS (-17.4±3.3 vs -21.0±2.7%, P<0.001) were significantly lower in ECS patients than controls. However, sarcoidosis showed a significantly larger effect on LV-GLS (SMD: -1.26, 95%CI -1.61,-0.91, P<0.001) rather than on LVEF (SMD: -0.51, 95%CI -0.83,-0.20, P=0.001). Substantial heterogeneity was found for the studies that assessed LV-GLS (I2=86.4%) and LVEF (I2=85.3%). Egger's test gave a P-value of 0.24 for LV-GLS and 0.32 for LVEF assessment, indicating no publication bias. On meta-regression analysis, none of the moderators was significantly associated with effect modification for both LV-GLS and LVEF (all P <0.05). In patients without overt heart disease, the effect of sarcoidosis on LV-GLS is significantly greater than on LVEF. STE analysis should be implemented in clinical practice for the early detection of myocardial involvement in ECS patients.

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.

Cardiac sarcoidosis treated with nonsteroidal immunosuppressive therapy

BackgroundNonsteroidal 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. MethodsFrom 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. ResultsNonsteroidal 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. ConclusionsNonsteroidal immunosuppressive therapy may be a possible treatment option for patients who are not stabilized with corticosteroids alone or develop corticosteroid side effects.

The usefulness of speckle tracking echocardiography for the prediction of cardiac involvement in patients with biopsy-proven sarcoidosis.

Cardiac sarcoidosis (CS) is commonly diagnosed based on clinical criteria and abnormalities in noninvasive imaging reported in patients with biopsy-proven extracardiac sarcoidosis. Electrocardiogram and two-dimensional echocardiography have a low sensitivity for CS detection. Cardiovascular magnetic resonance imaging (CMR) and positron emission tomography (PET) have limitations in terms of cost and availability. This study aimed to assess the usefulness of left ventricular longitudinal strain, measured using two-dimensional speckle tracking echocardiography (STE), for the prediction of late gadolinium enhancement (LGE) presence in CMR in patients with biopsy-proven sarcoidosis. A total of 119 patients with biopsy-proven extracardiac sarcoidosis were divided, according to the clinical criteria proposed by the 2014 Heart Rhythm Society expert consensus statement (HRS 2014), into two groups: 43 individuals with "probable cardiac sarcoidosis", CS(+) and 76 individuals without cardiac sarcoidosis, CS (-). Data from echocardiography, CMR, 12-lead ECG and 24h Holter monitoring were analyzed. Left ventricular global longitudinal strain (LV-GLS) was slightly reduced in the entire sarcoidosis group (-18.61±2.96), no difference between the CS (+) and CS (-) subgroups was found (-18.0%±3.2% and -18.9%±2.8%, respectively; p=.223). No cut-off value for LV-GLS was identified that could predict the presence of LGE. Segmental longitudinal strain impairment partially correlated with the presence of LGE on CMR. In our cohort of sarcoidosis patients, segmental longitudinal strain proved more helpful in the diagnostic process than LV-GLS. The ultimate role of STE in the diagnosis of CS remains to be established.

Abstract 13286: Co-Morbidities Clustering According to Imaging Diagnostic Criteria in Cardiac Sarcoidosis

Background: Cardiac sarcoidosis (CS) diagnostic guidelines updated in 2006 and in 2017 now include imaging findings of left ventricular (LV) dysfunction (LV EF &lt; 50%) on cardiac MRI and echocardiography as major diagnostic criterion. Limited data is available with regards to co-morbidities clustering in sarcoidosis and CS according to the 2017 updated guideline diagnostic criteria. Methods: A case control, single tertiary medical center study included 558 sarcoidosis patients with documented extracardiac sarcoidosis and completed electrocardiogram and/or cardiac MRI imaging. CS 2006 and 2017 diagnostic criteria and co-morbidity data were extracted from electronic charts and were available for comparison in 540 patients. Results: The total study population was composed of 52.7% (281/540) females, aged 58.9+/-12.6 years old, with diabetes mellitus (DM) present in 29.6% (160/540), HTN in 51.7% (279/540), CAD in 13.3% (72/540), moderate to severe aortic, mitral, or tricuspid valvular disease in 14.8% (80/540), ESRD in 2.4% (13/540), and COPD or asthma in 21.9% (118/540). There was a significant clustering of co-morbidities according to the imaging CS diagnostic criteria. Patients meeting the 2017 CS imaging criteria were found to be older (61.4+/-11.3 vs 58.8+/-12.8 years old in patients not meeting criteria, p=0.085), predominantly male (69.2%, (54/78) vs. 44.4%, (205/462), p&lt;0.001), and demonstrated significantly increased prevalence of DM (39.7%, (31/78) vs. 27.9%, (129/462), p=0.034), and valvular disease (33.3%, (26/78) vs. 11.7%, (54/462), respectively, p&lt;0.001). However, there were no statistically significant difference in the prevalence of HTN (60.3%, (47/78) vs. 50.2%, (232/462), p=0.101), ESRD (3.9%, (3/76) vs. 2.2%, (10/456), p=0.361), and COPD or asthma (19.2%, (15/78) vs. 22.3%, (103/462), p=0.544) in patients meeting 2017 CS criteria compared to those that did not. Conclusion: CS patients meeting 2017 imaging diagnostic criteria were found to have statistically significant clustering of the following co-morbidities: male gender, DM, and valvular disease. These findings underscore importance of comprehensive care in CS patients and warrant caution in assigning LV abnormalities observed on imaging solely to CS etiology.

The Role of Echocardiography in the Contemporary Diagnosis and Prognosis of Cardiac Sarcoidosis: A Comprehensive Review.

Cardiac sarcoidosis (CS) is a rare inflammatory disorder characterised by the presence of non-caseating granulomas within the myocardium. Contemporary studies have revealed that 25-30% of patients with systemic sarcoidosis have cardiac involvement, with detection rates increasing in the era of advanced cardiac imaging. The use of late gadolinium enhancement cardiac magnetic resonance and 18fluorodeoxy glucose positron emission tomography (FDG-PET) imaging has superseded endomyocardial biopsy for the diagnosis of CS. Echocardiography has historically been used as a screening tool with abnormalities triggering the need for advanced imaging, and as a tool to assess cardiac function. Regional wall thinning or aneurysm formation in a noncoronary distribution may indicate granuloma infiltration. Thinning of the basal septum in the setting of extracardiac sarcoidosis carries a high specificity for cardiac involvement. Abnormal myocardial echotexture and eccentric hypertrophy may be suggestive of active myocardial inflammation. The presence of right-ventricular involvement as indicated by free-wall aneurysms can mimic arrhythmogenic right-ventricular cardiomyopathy. More recently, the use of myocardial strain has increased the sensitivity of echocardiography in diagnosing cardiac involvement. Echocardiography is limited in prognostication, with impaired left-ventricular (LV) ejection fraction and LV dilatation being the only established independent predictors of mortality. More research is required to explore how advanced echocardiographic technologies can increase both the diagnostic sensitivity and prognostic ability of this modality in CS.

Quantitative myocardial T2 mapping adds value to Japanese circulation society diagnostic criteria for active cardiac sarcoidosis.

Noninvasive identification of active myocardial inflammation in patients with cardiac sarcoidosis plays a key role in management but remains elusive. T2 mapping is a proposed solution, but the added value of quantitative myocardial T2 mapping for active cardiac sarcoidosis is unknown. Retrospective cohort analysis of 56 sequential patients with biopsy-confirmed extracardiac sarcoidosis who underwent cardiac MRI for myocardial T2 mapping. The presence or absence of active myocardial inflammation in patients with CS was defined using a modified Japanese circulation society criteria within one month of MRI. Myocardial T2 values were obtained for the 16 standard American Heart Association left ventricular segments. The best model was selected using logistic regression. Receiver operating characteristic curves and dominance analysis were used to evaluate the diagnostic performance and variable importance. Of the 56 sarcoidosis patients included, 14 met criteria for active myocardial inflammation. Mean basal T2 value was the best performing model for the diagnosis of active myocardial inflammation in CS patients (pR2 = 0.493, AUC = 0.918, 95% CI 0.835-1). Mean basal T2 value > 50.8ms was the most accurate threshold (accuracy = 0.911). Mean basal T2 value + JCS criteria was significantly more accurate than JCS criteria alone (AUC = 0.981 vs. 0.887, p = 0.017). Quantitative regional T2 values are independent predictors of active myocardial inflammation in CS and may add additional discriminatory capability to JCS criteria for active disease.

PO-04-140 ARRHYTHMIA PRESENTATION AND MANAGEMENT IN PATIENTS WITH CLINICALLY DIAGNOSED CARDIAC SARCOIDOSIS

The diagnosis of cardiac sarcoidosis (CS) is often suspected based on clinical features when histologic evidence is lacking. While the arrhythmic characteristics of CS with histologic evidence meeting the 2014 Heart Rhythm Society criteria (definite or probable CS) have been extensively studied, the arrhythmic presentations and outcomes of clinical CS are less known. To compare arrhythmia manifestations, management, and outcomes between clinically diagnosed and definite/probable CS. We retrospectively analyzed a single-center referral cohort of patients with available electrophysiologic data stratified by CS category (definite/probable or clinical). Patients with clinical CS had no histologic evidence of cardiac or extracardiac sarcoidosis (biopsy negative or not performed) and the diagnosis was based on clinical course (unexplained sustained VT, Mobitz II, or complete AV block) and imaging features (patchy uptake on dedicated cardiac positron emission tomography or late gadolinium enhancement on cardiac magnetic resonance imaging). History of arrhythmias at index evaluation, arrhythmia management, and outcomes were comparatively assessed for patients with definite/probable CS compared to those with clinical CS. The cohort included 629 CS patients (median age 57 years, male 67%, NYHA class I-II 78%), including 12% definite, 54% probable, and 34% clinical CS patients. History of ventricular and supraventricular arrhythmias was more common in clinical CS compared to definite/probable CS (Figure). Accordingly, the rate of antiarrhythmic drug use was higher in clinical (33%) than definite/probable CS (22%, p=0.002). Median follow-up after index evaluation at our institution was 3.1y (IQR 4.2y). The number of incident VT/VF events per patient per year was greater in clinical CS than definite/probable CS (Figure). Catheter ablations for ventricular arrhythmia were more frequent in clinical CS (27%) than definite/probable CS (9%, p<0.001). Patients with clinical CS exhibited a more severe arrhythmic phenotype compared to patients with definite/probable CS. These findings have implications for the management and prognosis of CS patients within this common clinical category.

Linking cardiac and extracardiac sarcoidosis and their clinical outcome: 18F-FDG PET/CT analysis in patients with systemic cardiac sarcoidosis.

To clarify the link between cardiac sarcoidosis (CS) and extra-CS (ECS) in systemic CS (SCS) patients in terms of extent and clinical outcome by serial FDG-PET/CT. Thirty-five SCS patients treated for > 2years were enrolled in this study. In the overall analysis, patient-based comparisons of the complete resolution (CR) and recurrence rate between CS and ECS lesions were performed. Then, subgroup analyses were performed according to the extent (mono- vs. multi-organ ECS group) and clinical outcome (stable vs. unstable ECS group) of ECS. Pre-treatment cardiac FDG uptake was compared between the mono- and multi-organ ECS groups. The rates of CR, recurrence, and major adverse cardiac events (MACE) were compared between the two groups. The CR rate was significantly higher in CS than ECS lesions [77.1% (27/35) vs. 48.5% (17/35), p = 0.01], whereas recurrence rates were similar between CS and ECS [40.7% (11/27) vs. 58.8% (10/17)]. Both the mono- and multi-organ ECS groups showed similar SUVmax, cardiac metabolic volume, and cardiac metabolic activity in the pre-treatment condition. The CR rates were similar between the mono- and multi-organ ECS groups [71.4% (15/21) vs. 85.7% (12/14)], but the recurrence rate was significantly lower in the multi-organ ECS group [60.0% (9/15) vs. 16.7% (2/12), p = 0.02]. The CR [71.4% (5/7) vs. 78.6% (22/28)] and recurrence rates [60.0% (3/5) vs. 36.3% (8/22)] were not significantly different between the stable and unstable ECS groups. The occurrence of MACE was also not significantly different between the mono- and multi-organ ECS groups [19.0% (4/21) vs. 28.6% (4/14)] or between the stable and unstable ECS groups [42.9% (3/7) vs. 17.8% (5/28)]. CS lesions respond to treatment better than ECS lesions, and the extent and clinical outcome of ECS lesion are not linked with those of CS lesions.

Incidence of Atrial Fibrillation as the Initial Manifestation of Cardiac Sarcoidosis: Insights From a Catheter Ablation Registry

BackgroundCardiac sarcoidosis (CS) is a rare form of arrhythmogenic cardiomyopathy; a delayed diagnosis can lead to significant consequences. Patients with clinically manifest CS often have minimal extracardiac involvement and thus frequently present initially to cardiology. Indeed, certain specific arrhythmic scenarios should trigger investigations for undiagnosed CS. Atrial fibrillation (AF) has been described as one of the presenting features of CS; however, the incidence of this presentation is not known. MethodsAt our institution, cardiac computerized tomography is routinely performed prior to catheter ablation for AF. Noncardiac incidental findings are described by radiologists and are followed-up by interval investigations. We systematically reviewed noncardiac reports from 1574 consecutive patients in our prospective AF ablation registry. Specifically, we used text-scraping techniques to search on the following keywords: “adenopathy” and “sarcoidosis.” Detailed chart review of identified cases was then performed to evaluate results of interval investigations and assess long-term outcomes. ResultsTwenty of 1574 patients (1.3%) had noncardiac reports containing “adenopathy” and/or “sarcoidosis.” After interval imaging and a follow-up period averaging 60 ± 35 months, only 2 patients of 1574 (0.13%) were diagnosed with CS. Four of 20 (20%) had a previous history of extracardiac sarcoidosis, and another 1 of 20 (5%) was subsequently diagnosed with extracardiac sarcoidosis. However, none of these 5 patients had evidence of cardiac involvement. ConclusionsCS is a rare finding among patients undergoing a first-time AF ablation. Our findings suggest that AF is an uncommon initial presentation of CS. Thus, investigations for CS in patients with AF are not warranted routinely, unless additional suggestive clinical features are present.

Challenges of cardiac sarcoidosis.

Sarcoidosis is a multisystem granulomatosis of unknown origin, which can involve almost any organ. Most frequently the disease involves the lungs and mediastinal lymph nodes, but it can affect the skin, the eyes, nervous system, the heart, kidneys, joints, muscles, calcium metabolism, and probably any other anecdotical organ involvement. Cardiac sarcoidosis is one of the most challenging involvements, as it can lead to cardiac mortality and morbidity, and also because the diagnosis may be difficult. With no specific symptoms, cardiac sarcoidosis may be difficult to suspect in a patient with no previous extra-cardiac sarcoidosis diagnosis. This manuscript reviews the current knowledge of the diagnosis and decision to treat cardiac sarcoidosis, and illustrates the information with a case presentation of a young adult with no risk factors, no previous diagnosis of sarcoidosis, and with cardiac symptoms impairing his quality of life.

Abstract 11874: Clinical Outcomes and Predictors of Long-Term Survival in Patients With and Without Extra-Cardiac Sarcoidosis Using Machine Learning: A Swedish Multicenter Study

Introduction: Sarcoidosis is a systemic disease but cardiac involvement (CS) as first organ manifestation is not uncommon. Hypothesis: Patients with CS as the first manifestation of sarcoidosis has worse outcome compared to patients with known extra cardiac sarcoidosis (ECS) Methods: A retrospective cohort of 141 patients with CS enrolled at two Swedish university hospitals was studied. Presentation, treatment and outcomes of de novo CS and previous-known extra-CS group (ESC) were compared. Survival free time of primary composite outcome (ventricular tachycardia /ventricular fibrillation , heart transplantation , or death) was assessed. Using a data-driven approach with a machine learning (ML) algorithm, we studied the relative importance of several factors Results: 62 de novo CS patients and 79 with known ECS at time of cardiac manifestation. No difference in the demographics was observed between the groups. De novo CS patients showed more advanced NYHA class (p=0.02) as well as higher circulating levels of natriuretic peptides (p&lt;0.001), troponins (p&lt;0.001). Imaging findings were similar between the two groups. During a median (IQR) follow-up time was 28 [16-80] months, the composite end-point occurred in 37% of patients with CS as first clinical manifestation and in 20% of patients with ECS. Kaplan-Meier analysis showed that event-free survival was significantly worse in patients with de novo CS (p&lt;0.001) ( Fig. 1 ). The top four factors to predict event-free survival in order of importance were: tricuspid annular plane systolic excursion (TAPSE), Cardiac Index (CI), Right Ventricular (RV) ejection fraction, previous known ECS Conclusions: Patients presenting with CS as their first recognized organ manifestation of disease display worse outcomes compared to patients with previous-known ECS. Using machine learning, we show that the most important predictor of survival in CS is TAPSE, following by CI, RV ejection fraction and previous known ECS

Abstract 11404: Phenotyping Cardiac Sarcoidosis With PET/MR: Imaging Characteristics, Treatment Response, and Outcomes of Isolated Cardiac Sarcoidosis versus Extra-Cardiac Sarcoidosis With Cardiac Involvement

Introduction: Diagnosing cardiac sarcoidosis (CS) remains challenging due to limitations of available clinical criteria and low yield of endomyocardial biopsy. PET-MR imaging allows for multidimensional non-invasive assessment of suspected CS. However, the true diagnostic and prognostic impact of these modalities require further exploration. Hypothesis: PET-MR can phenotype CS and its associated treatment response. Methods: We identified 100 patients with known or suspected CS (52±1.8 years, 66% male) who underwent PET-MR imaging: 38 individuals with isolated CS (ICS) (50±1.8 years, 65% male) and 62 subjects with CS and biopsy-proven extra-cardiac sarcoidosis (ECS) (52±2.4 years, 66% male). A subset of 56 patients (24 ICS and 32 ECS) was classified as the phenotype cohort if they were: 1) treatment naïve, 2) started on immunosuppressant treatment after PET, and 3) had follow up PET to assess treatment response, which was graded as complete (CTR), partial (PTR), and no response (NR). PET-MR phenotype was graded as combined imaging findings suggestive of CS (Figure 1A), equivocal for CS, and not suggestive of CS. Major adverse cardiovascular events (MACE) were defined as sustained ventricular tachycardia, ventricular fibrillation, heart transplantation and/or cardiac death at 1 year. Results: From the ICS phenotype cohort, PET-MR findings suggestive of CS were present in 100% (8/8) of patients with biopsy-proven ICS vs. 56% (9/16) of subjects without biopsy (P=0.03). CTR/PTR rates were overall higher in ECS compared to ICS (81% vs. 58%; P=0.06), and in patients with PET-MR suggestive of CS (P=0.01; Figure 1B), in both the ECS (P=0.04) and ICS (P=0.08) groups. MACE events were significantly greater in ICS vs ECS (HR 3.9 [1.6 - 9.7]; P=0.003), and in patients with PET-MR suggestive of CS (HR 8.2 [1.1 - 62]; P=0.04; Figure 1C). Conclusion: PET-MR can identify phenotypes of CS which have distinct disease manifestations, treatment response rates, and clinical outcomes.

Abstract 9231: Contribution of Echocardiographic Abnormalities to Cardiac Sarcoidosis Diagnosis According to Past and Contemporary Diagnostic Criteria

Introduction: Cardiac sarcoidosis (CS) guidelines were originally published in 1993 by the Japanese Ministry of Health and Welfare (JMHW) and updated in 2006 and 2017. The updated guidelines consider several echocardiographic abnormalities as major diagnostic CS criteria. Contribution of this change to CS diagnosis has not been well investigated. Methods: A case control, single tertiary medical center study included 571 sarcoidosis patients (52.7% females, 58.9+/-12.6 years old) with documented extracardiac sarcoidosis and completed electrocardiogram and imaging assessment. Echocardiographic abnormalities consistent with CS were left ventricular (LV) dilation (LVD), LV aneurysms (LVA), LV wall motion abnormalities (LVWMA), LV hypertrophy (LVH), right ventricular hypertrophy (RVH), focal thinning of the anterior septum, and LV ejection fraction (EF) less than 50%. Frequency of echocardiographic abnormalities according to 1993, 2006 and 2017 CS diagnostic criteria were compared. Results: Most common echocardiographic abnormalities were reduced LVEF and LVH, occurring in more than 14 and 12% of CS patients. LVD and LVWMA occurred 8 and 7%, followed by uncommon RVH, LVA, and septal thinning (Table). Despite being common in CS, only LVD, LWMA, and reduced LVEF significantly (p&lt;0.001) contributed to the CS diagnosis, while LVH did not (p=0.609). Alternatively, while LV aneurysm and septal thinning were uncommon, they significantly contributed to CS diagnosis (p&lt;0.001). RVH was very uncommon and did not affect CS diagnosis. Conclusions: CS diagnostic criteria highlights the importance of cardiac imaging in suspected CS. Commonly seen echocardiographic findings of CS include EF&lt;50%, LVWMA, and LV dilation significantly contribute to CS diagnosis. Of less commonly seen abnormalities, RVH utility appears to be low, while LVA and septal thinning may be helpful. Echocardiography remains an integral part in evaluating individuals with suspected CS.

ACE values in the diagnosis of sarcoidosis

Abstract Background In clinical practice, we often encounter the patients with sarcoidosis showing relatively high in the normal range of serum angiotensin-converting enzyme (ACE) value. Purpose We aimed to examine the serum ACE value of the patients with sarcoidosis and determine the new cut-off value for detecting the patients with sarcoidosis. Methods and results We retrospectively examined all 3781 subjects (51.1% men, 60.1±17.0 y.o.) in whom ACE was measured for any reasons including suspected sarcoidosis between 2009 and 2020 in our hospital. Of 293 patients with sarcoidosis, 101, 212, 84, and 88 were diagnosed as sarcoidosis in heart, lung, skin, and eyes, respectively. After excluding 477 patients taking ACE inhibitor and/or immunosuppression agent or those with any diseases affecting serum ACE levels, we analyzed 3304 subjects including 215 with sarcoidosis. Serum ACEs were 19.6 IU/L [IQR, 15.1–31.5] in the subjects with sarcoidosis and 10.7 [8.4–16.5] in those without (P&amp;lt;0.01). In ROC curve analysis of ACE for diagnosis of sarcoidosis, the cut-off point was 14.7 IU/L and the AUC was 0.865. When we used the current cut-off of 21.4 or new cut-off value of 14.7, sensitivity, specificity, PPV, NPV, and accuracy were shown in Table. Finally, they were divided into four groups based on the presence of cardiac and/or extra cardiac sarcoidosis, ACE values in Group A, B, C, and D were 17.9, 20.9, 18.6, and 10.7, respectively (Figure 1). Conclusion(s) In the current cut-off value of serum ACE, sensitivity for detecting sarcoidosis was comparatively low, though positive predictive value was low when the new-cut-off value was used in our study. Further examinations may be needed for the patients suspected sarcoidosis with relatively high ACE in the normal range. Funding Acknowledgement Type of funding sources: None.

Impact of cardiac resynchronisation therapy in patients with cardiac sarcoidosis

Abstract Background Implantation of a device is usually required in cardiac sarcoidosis (CS) patients presenting with advanced conduction abnormalities or ventricular arrhythmias. A cardiac resynchronisation therapy (CRT) device is often chosen in patients with concomitant left ventricular systolic impairment. The role of CRT in CS is not well established. Purpose To describe the cohort of CS patients with CRT device in situ in our hospital focusing on the short-term effect in serial echocardiography and long-term outcomes on morbidity and mortality. Methods All consecutive CS patients with a CRT device in situ were identified in our CS database (2005–2022). A confident CS diagnosis was provided after review of all relevant clinical and imaging baseline data in our CS multi-disciplinary meeting and a consensus decision for CRT-D implantation was made based on international guidelines. All patients were followed up for at least 6 months with serial echocardiography. Serial data regarding symptoms, rhythm disturbance and echocardiographic parameters were obtained and comparisons were performed using Wilcoxon signed rank test. Results A total of 51 CS patients with CRT-D were identified (mean age: 57±10 years old). Patients were male predominant (64.7%) and Caucasian in origin (86.2%). Extra-cardiac sarcoidosis was confirmed histologically in 33 (64.7%) patients. The prevalence of smoking, diabetes, hypertension and ischaemic heart disease was 27.5%, 21.6%, 49.0% and 7.8% respectively. At the time of device implantation or during follow-up, 43 (84.3%) patients were found to have active cardiac sarcoidosis on cardiac PET. Post CRT implantation there was a significant difference in LV ejection fraction (35.9±15.0% vs 42.2±14.1%, p&amp;lt;0.001), LV end-systolic diameter (4.90±1.46 cm vs 4.62±1.32 cm, p=0.012) and LV end-diastolic diameter (5.99±1.18 cm vs 5.66±1.06 cm, p&amp;lt;0.001). No significant changes were observed in the right ventricular function (p=0.09) and severity of mitral regurgitation (p=0.40). There was one patient who experienced acute heart failure decompensation admission within six months of CRT-D implantation. The New York Heart Association (NYHA) class improved in 26 patients (51.0%), worsened in 4 (7.8%) patients and remained the same in 21 (41.2%) patients at 6 months post CRT-implantation. During the mean follow up of 47.6 months, the composite end-point of death and cardiac transplantation was reached in 9 (17.6%) patients (8 deaths and 1 cardiac transplantation). 5 patients had major complications including a large haematoma, a small atrio-septal defect, haemothorax, device associated endocarditis and lead fracture. Minor wound infections were seen in 3 patients and 4 patients received inappropriate shock or anti-tachycardia pacing. Conclusions CRT in cardiac sarcoidosis patients is associated with short-term improvement in LV remodelling and functional status but over a four year follow up, morbidity and mortality are common. Funding Acknowledgement Type of funding sources: None.

Cardiac resynchronization therapy response in cardiac sarcoidosis.

Cardiac sarcoidosis (CS) is a nonischemic cardiomyopathy (NICM) characterized by infiltration of noncaseating granulomas involving the heart with highly variable clinical manifestations that can include conduction abnormalities and systolic heart failure. Cardiac resynchronization therapy (CRT) has shown significant promise in NICM, though little is known about its efficacy in patients with CS. To determine if CRT improved cardiac remodeling in patients with CS. We retrospectively reviewed all patients with a clinical or histological diagnosis of CS who underwent CRT implantation at the Mayo Clinic enterprise from 2000 to 2021. Baseline characteristics, imaging parameters, heart failure hospitalizations and need for advanced therapies, and major adverse cardiac events (MACE) were assessed. Our cohort was comprised of 55 patients with 61.8% male and a mean age of 58.7 ± 10.9 years. Eighteen (32.7%) patients had definite CS, 21 (38.2%) had probable CS, while 16 (29.1%) had presumed CS, and 26 (47.3%) with extracardiac sarcoidosis. The majority underwent CRT-D implantation (n = 52, 94.5%) and 3 (5.5%) underwent CRT-P implantation with 67.3% of implanted devices being upgrades from prior pacemakers or implantable cardioverter defibrillators. At 6 months postimplantation there was no significant improvement in ejection fraction (34.8 ± 10.9% vs. 37.7 ± 14.2%, p = .331) or left ventricular end-diastolic diameter (58.5 ± 10.2 vs. 57.5 ± 8.1 mm, p = .236), though mild improvement in left ventricular end systolic diameter (49.1 ± 9.9 vs. 45.7± 9.9 mm, p < .0001). Within the first 6 months postimplantation, 5 (9.1%) patients sustained a heart failure hospitalization. At a mean follow-up of 4.1± 3.7 years, 14 (25.5%) patients experienced a heart failure hospitalization, 11 (20.0%) underwent cardiac transplantation, 1 (1.8%) underwent left ventricular assist device implantation and 7 (12.7%) patients died. Our findings suggest variable response to CRT in patients with CS with no overall improvement in ventricular function within 6 months and a substantial proportion of patients progressing to advanced heart failure therapies.

Cardiac Magnetic Resonance Imaging-Based Screening for Cardiac Sarcoidosis in Patients With Atrioventricular Block Requiring Temporary Pacing.

BackgroundSome myocardial diseases, such as cardiac sarcoidosis, predispose to complete atrioventricular block. The European Society of Cardiology Guidelines on cardiac pacing in 2021 recommend myocardial disease screening in patients with conduction disorder requiring pacemaker with multimodality imaging, including cardiac magnetic resonance (CMR) imaging. The ability of CMR imaging to detect myocardial disease in patients with a temporary pacing wire is not well documented.Methods and ResultsOur myocardial disease screening protocol is based on using an active fixation pacing lead connected to a reusable extracorporeal pacing generator (temporary permanent pacemaker) as a bridge to a permanent pacemaker. From 2011 to 2019, we identified 17 patients from our CMR database who underwent CMR imaging with a temporary permanent pacemaker for atrioventricular block. We analyzed their clinical presentations, CMR data, and pacemaker therapy. All CMRs were performed without adverse events. Pacing leads induced minor artifacts to the septal myocardial segments. The extent of late gadolinium enhancement in CMR imaging was used to screen patients for the presence of myocardial disease. Patients with evidence of late gadolinium enhancement underwent endomyocardial biopsy. If considered clinically indicated, also 18‐F‐fluorodeoxyglucose positron emission tomography and extracardiac tissue biopsy were performed if sarcoidosis was suspected. Eventually, 8 of 17 patients (47.1%) were diagnosed with histologically confirmed granulomatous inflammatory cardiac disease. Importantly, only 1 had a previously diagnosed extracardiac sarcoidosis at the time of presentation with high‐degree atrioventricular block.ConclusionsCMR imaging with temporary permanent pacemaker protocol is an effective and safe early screening tool for myocardial disease in patients presenting with atrioventricular block requiring immediate, continuous pacing for bradycardia.

Cardiac sarcoidosis outcome differences: A comparison of patients with de novo cardiac versus known extracardiac sarcoidosis at presentation

BackgroundSarcoidosis is a systemic disease characterized by granulomatous inflammation. Cardiac involvement is associated with increased morbidity. However, differences in clinical characteristics and outcomes based on initial sarcoidosis organ manifestation in patients with cardiac sarcoidosis (CS) have not been described. MethodsA retrospective cohort of 252 patients with CS at an urban, quaternary medical center was studied. Presentation, treatment and outcomes of de novo CS and prior ECS groups were compared. Survival free of primary composite outcome (left ventricular assist device implantation, orthotopic heart transplantation (OHT), or death) was assessed. ResultsThere were 124 de novo CS patients and 128 with prior ECS at time of CS diagnosis. De novo CS patients were younger at CS diagnosis (p = 0.020). De novo CS patients had a more advanced cardiac presentation: lower left ventricular ejection fraction (LVEF) (p < 0.001), more frequent sustained ventricular arrhythmias (VA) (p = 0.001), and complete heart block (p = 0.001). During follow-up, new VA (p < 0.001), ventricular tachycardia ablation (p < 0.001), and OHT (p = 0.003) were more common in the de novo CS group. Outcome free survival was significantly shorter for de novo CS patients (p = 0.005), with increased hazard of primary composite outcome (p = 0.034) and development of new VA (p = 0.027) when compared to ECS patients. Overall mortality was similar between groups. ConclusionPatients presenting with CS as their first recognized organ manifestation of sarcoidosis have an increased risk of adverse cardiac outcomes as compared to those with a prior history of ECS. Improved awareness and diagnosis of CS is warranted for earlier recognition.

PO-656-01 PSEUDOINFARCT PATTERN IN CARDIAC SARCOIDOSIS: CORRELATION OF Q WAVES ON 12-LEAD ELECTROCARDIOGRAM WITH LATE GADOLINIUM ENHANCEMENT ON CARDIAC MRI AND PREDICTION OF VENTRICULAR TACHYARRHYTHMIAS

Screening for cardiac sarcoidosis (CS) with a 12-lead ECG is recommended in all patients with extra-cardiac sarcoidosis. Previous studies have characterized the poor sensitivity of AV conduction disease in screening for CS, but few have evaluated the presence of a pseudoinfarct pattern as a screening tool and a possible predictor of ventricular arrhythmias (VA).