Cardiac sarcoidosis: A review on the work-up and management

Open Medicine ◽  
2013 ◽  
Vol 8 (1) ◽  
pp. 107-116
Author(s):  
Boris Solun ◽  
Dana Marcoviciu ◽  
Yulia Belnik ◽  
Tamar Azran ◽  
Dror Dicker ◽  
...  
Keyword(s):  
Sudden Death ◽  
Cardiac Sarcoidosis ◽  
Ventricular Tachyarrhythmia ◽  
Clinical Manifestations ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Diagnostic Tools ◽  
Unknown Etiology ◽  
Ventricular Ejection Fraction ◽  
Electrocardiographic Monitoring

AbstractSarcoidosis is a multisystem granulomatous disease of unknown etiology and with variable presentation. Skin, lymph nodes, lungs, eyes and the central nervous system are mostly involved. Cardiac sarcoidosis (CS) is a rare condition with clinical manifestations in about 5% of patients. Since it increases the risk of acute cardiac failure, ventricular arrhythmia, conduction disturbances and even sudden death, it aggravates markedly the prognosis. The early diagnosis of CS is difficult, requiring the use of diagnostic tools such as electrocardiographic monitoring, two-dimensional echocardiography, radionuclide scan, cardiac magnetic resonance imaging, positron emission tomography and endomyocardial biopsy. Once the diagnosis of CS is established, there is a need for early corticosteroids treatment, with or without immunosuppressive therapy, to prevent deterioration of cardiac function. In patients with refractory ventricular tachyarrhythmia, markedly reduced left ventricular ejection fraction and high risk of sudden death, prophylactic insertion of a pacemaker or implantable defibrillator is recommended. We had the opportunity to treat a patient with CS and to review the currently accepted diagnostic and treatment approach.

scholarly journals Combined simultaneous FDG-PET/MRI with T1 and T2 mapping as an imaging biomarker for the diagnosis and prognosis of suspected cardiac sarcoidosis

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Edward Cheung ◽  
Sarah Ahmad ◽  
Matthew Aitken ◽  
Rosanna Chan ◽  
Robert M. Iwanochko ◽  
...  
Keyword(s):  
Fdg Pet ◽  
Cardiac Sarcoidosis ◽  
T2 Mapping ◽  
Ventricular Tachyarrhythmia ◽  
Prognostic Significance ◽  
Final Diagnosis ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Fdg Uptake

Abstract Purpose To evaluate the diagnostic and prognostic significance of combined cardiac 18F-fluorodeoxyglucose (FDG) PET/MRI with T1/T2 mapping in the evaluation of suspected cardiac sarcoidosis. Methods Patients with suspected cardiac sarcoidosis were prospectively enrolled for cardiac 18F-FDG PET/MRI, including late gadolinium enhancement (LGE) and T1/T2 mapping with calculation of extracellular volume (ECV). The final diagnosis of cardiac sarcoidosis was established using modified JMHW guidelines. Major adverse cardiac events (MACE) were assessed as a composite of cardiovascular death, ventricular tachyarrhythmia, bradyarrhythmia, cardiac transplantation or heart failure. Statistical analysis included Cox proportional hazard models. Results Forty-two patients (53 ± 13 years, 67% male) were evaluated, 13 (31%) with a final diagnosis of cardiac sarcoidosis. Among patients with cardiac sarcoidosis, 100% of patients had at least one abnormality on PET/MRI: FDG uptake in 69%, LGE in 100%, elevated T1 and ECV in 100%, and elevated T2 in 46%. FDG uptake co-localized with LGE in 69% of patients with cardiac sarcoidosis compared to 24% of those without, p = 0.014. Diagnostic specificity for cardiac sarcoidosis was highest for FDG uptake (69%), elevated T2 (79%), and FDG uptake co-localizing with LGE (76%). Diagnostic sensitivity was highest for LGE, elevated T1 and ECV (100%). After median follow-up duration of 634 days, 13 patients experienced MACE. All patients who experienced MACE had LGE, elevated T1 and elevated ECV. FDG uptake (HR 14.7, p = 0.002), elevated T2 (HR 9.0, p = 0.002) and native T1 (HR 1.1 per 10 ms increase, p = 0.044) were significant predictors of MACE even after adjusting for left ventricular ejection fraction and immune suppression treatment. The presence of FDG uptake co-localizing with LGE had the highest diagnostic performance overall (AUC 0.73) and was the best predictor of MACE based on model goodness of fit (HR 14.9, p = 0.001). Conclusions Combined cardiac FDG-PET/MRI with T1/T2 mapping provides complementary diagnostic information and predicts MACE in patients with suspected cardiac sarcoidosis.

Mode of Death in Patients With Heart Failure and Preserved Ejection Fraction: Insights From PARAGON-HF Trial

2021 ◽  
Author(s):  
Akshay S. Desai ◽  
Muthiah Vaduganathan ◽  
John G. Cleland ◽  
Brian L. Claggett ◽  
Ebrahim Barkoudah ◽  
...  
Keyword(s):  
Heart Failure ◽  
Left Ventricular Ejection Fraction ◽  
Sudden Death ◽  
Ejection Fraction ◽  
Cardiovascular Death ◽  
Left Ventricular ◽  
Ventricular Ejection ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Mode Of Death

Background: Patients with heart failure (HF) and preserved left ventricular ejection fraction comprise a heterogeneous group including some with mildly reduced EF. We hypothesized that mode of death differs by EF in ambulatory patients with HF and preserved left ventricular ejection fraction. Methods: PARAGON-HF trial (Prospective Comparison of Angiotensin Receptor–Neprilysin Inhibitor With Angiotensin-Receptor Blocker Global Outcomes in Heart Failure With Preserved Ejection Fraction) compared clinical outcomes in 4796 patients with chronic HF and EF ≥45% randomly assigned to sacubitril/valsartan or valsartan. We examined the mode of death in relation to baseline EF in logistic regression models and the effect of randomized treatment on cause-specific death in Cox regression models. Nonlinear relationships with continuous EF were modelled using quadratic and cubic terms. Results: Of 691 deaths during the trial, 416 (60%) were ascribed to cardiovascular, 220 (32%) to noncardiovascular, and 55 (8%) to unknown causes. Of cardiovascular deaths, 154 (37%) were due to sudden death, 118 (28%) were due to HF, 35 (8%) to stroke, 27 (6%) to myocardial infarction, and 82 (20%) to other cardiovascular causes. Rates of all-cause, cardiovascular, and sudden death were higher in those with lower left ventricular ejection fraction (all P <0.001), while rates of non-cardiovascular death were greater in patients with higher EF. Sacubitril/valsartan did not reduce overall death, cardiovascular death, or sudden death compared with valsartan, irrespective of baseline EF (all P for interaction >0.30). Conclusions: Among patients with HF and preserved left ventricular ejection fraction enrolled in PARAGON-HF, the proportion of cardiovascular and sudden death were higher in those with lower left ventricular EF, and the proportion of noncardiovascular death rose with EF. Regardless of EF, sacubitril/valsartan did not reduce death from any cause compared with valsartan. REGISTRATION: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT01920711.

Beyond ejection fraction: Novel clinical approaches towards sudden cardiac death risk stratification in patients with dilated cardiomyopathy

2021 ◽  
Vol 17 ◽  
Author(s):  
Issa Pour-Ghaz ◽  
Mark Heckle ◽  
Ikechukwu Ifedili ◽  
Sharif Kayali ◽  
Christopher Nance ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Ejection Fraction ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Ventricular Tachyarrhythmia ◽  
Total Mortality ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Icd Therapy

: Implantable cardioverter-defibrillator (ICD) therapy is indicated for patients at risk for sudden cardiac death due to ventricular tachyarrhythmia. The most commonly used risk stratification algorithms use left ventricular ejection fraction (LVEF) to determine which patients qualify for ICD therapy, even though LVEF is a better marker of total mortality than ventricular tachyarrhythmias mortality. This review evaluates imaging tools and novel biomarkers proposed for better risk stratifying arrhythmic substrate, thereby identifying optimal ICD therapy candidates.

Abstract 5935: Influence of Recent Advances in the Management of Heart Failure and Ventricular Arrhythmias on Survival in Patients with Cardiac Sarcoidosis

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Yoshikazu Yazaki ◽  
Mitsuaki Horigome ◽  
Kazunori Aizawa ◽  
Takeshi Tomita ◽  
Hiroki Kasai ◽  
...  
Keyword(s):  
Heart Failure ◽  
Ventricular Tachycardia ◽  
Cardiac Sarcoidosis ◽  
Severe Heart Failure ◽  
Left Ventricular ◽  
Cardiac Resynchronization ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Predictors Of Mortality ◽  
Recent Advances

Background : We previously described severity of heart failure and ventricular tachycardia (VT) as independent predictors of mortality in patients with cardiac sarcoidosis (CS). Medical treatment for chronic heart failure has been established over the last few decades. Prophylactic use of implantable cardioverter defibrillator (ICD) and cardiac resynchronization therapy (CRT or CRT-D) have been introduced in patients with severe heart failure. We therefore hypothesized that the prognosis of CS improves due to such advances in the management of heart failure and VT. Methods : To confirm our hypothesis, we analyzed 43 CS patients diagnosed between 1988 and 2006 and treated with corticosteroids. We classified two sequential referral patients diagnosed between 1988 and 1997 (n=19) and between 1998 and 2006 (n=24), and compared treatment and prognosis between the two cohorts. Results : Left ventricular ejection fraction (LVEF) and dimensions were similar between the two cohorts. Although age in the 1988–1997 referral cohort was significantly younger than that in the 1998–2006 referral cohort (54±14years versus 62±10years, p<0.05), survival in the earlier cohort was significantly worse (log-rank=4.41, p<0.05). The 1- and 5-year mortality rates were 88% and 71% in the 1988–1997 referral cohort, and 96% and 92% in the 1998–2006 referral cohort, respectively. The 1998–2006 referral cohort showed significantly higher incidence of ICD or CRT-D implantation (29% versus 6%, p<0.05), β-blocker use (46% versus 6%, p<0.01) and addition of methotrexate (21% versus 0%, p<0.05), and increased maintenance dose (7.0±1.9mg/day versus 5.0±0.9mg/day, p<0.01) compared to the 1988–1997 referral cohort. Multivariate analysis including age, LVEF, and sustained ventricular tachycardia (sVT) identified diagnosis between 1988 and 1997 (hazard ratio [HR]: 19.8, p<0.01) and LVEF (HR: 0.83/1% increase, p<0.01) as independent predictors of mortality. Conclusions : Survival in the recent CS patients is significantly better than previously described. Recent advances in the device therapies and medical treatments including modified immunosuppression alter the clinical outcome in patients with CS.

P1807Manifestations and outcome of cardiac sarcoidosis - does gender matter?

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
P Simonen ◽  
J Lehtonen ◽  
M Kupari
Keyword(s):  
Cardiac Sarcoidosis ◽  
Hot Spot ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Diagnostic Process ◽  
Term Outcome ◽  
Ventricular Ejection Fraction ◽  
Long Term Outcome

Abstract Background Sarcoidosis is characterized by the formation of inflammatory epithelioid-cell granulomas in various organs with cardiac involvement as its most ominous manifestation. A female preponderance in the prevalence of cardiac sarcoidosis (CS) is well known but other possible gender differences remain poorly studied. Purpose We set out to evaluate gender-related differences in the manifestations and long-term outcome of CS. Methods We reviewed the history, diagnostic procedures, details of treatment and outcome of 158 consecutive patients with histologically confirmed CS diagnosis between 1988 and 2017 at our hospital. Follow-up data were collected up to the end of 2018. Results The study population consisted of 51 men and 107 women (68%). At presentation, men were younger than women (mean age 47 years vs 51 years, p=0.045) and had more often a history of pre-existing extracardiac sarcoidosis (25% vs 10%, p=0.013). Isolated CS remained less common in men even after the complete diagnostic process (50% vs 75%, p=0.001). The main presenting CS manifestations were atrioventricular block, ventricular tachyarrhythmias and heart failure in 39%, 30% and 18% of men vs in 54%, 23% and 17% of women, respectively (p=0.183). Left ventricular ejection fraction at presentation averaged 49±11% in men and 49±13% in women (p=0.845). Troponin T was elevated more often in men at the presentation (46% vs 26%, p=0.024). At magnetic resonance imaging, pathological myocardial late gadolinium enhancement was observed in 87% of men and 84% of women (p=0.615). Myocardial “hot spot” at 18-F fluorodeoxyglucose positron emission tomography was also equally common (87% in men, 92% in women, p=0.468). An intracardiac cardioverter-defibrillator was implanted in 78% of men and 75% of women (p=0.693) and nearly all patients (99%, no gender difference) received immunosuppressive therapy. During the mean follow-up of 64 months, 10 of 51 men versus 30 of 107 women either died of a cardiac cause, suffered an aborted sudden cardiac death or underwent transplantation. The composite event-free survival did not differ between genders (Figure 1. Log-rank p=0.852). Conclusions Two thirds of CS patients are women. At disease presentation, women are older than men and their sarcoidosis is more often isolated to the heart but the clinical manifestations, diagnostic findings and long-term outcome are comparable in the two genders.

Cardiomyopathy induced by cardiac Gs alpha overexpression

1997 ◽  
Vol 272 (1) ◽  
pp. H585-H589 ◽  
Author(s):  
M. Iwase ◽  
M. Uechi ◽  
D. E. Vatner ◽  
K. Asai ◽  
R. P. Shannon ◽  
...  
Keyword(s):  
Extended Period ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Sympathetic Stimulation ◽  
Ventricular Ejection Fraction ◽  
Heart Rates ◽  
Electrocardiographic Monitoring ◽  
Stimulatory G Protein ◽  
G Protein Alpha Subunit ◽  
Gs Alpha

The goal of this study was to determine whether chronic endogenous sympathetic stimulation resulting from the overexpression of cardiac stimulatory G protein alpha subunit (Gs alpha) in transgenic mice (15.3 +/- 0.1 mo old) resulted in a clinical picture of cardiomyopathy. The left ventricular ejection fraction, measured by echocardiography, was reduced in older mice with Gs alpha overexpression (50.4 +/- 5.4%) compared with age-matched control mice (70.9 +/- 1.6%; P < 0.05). When ejection fractions were compared at similar heart rates, the Gs alpha mice exhibited a greater left ventricular end-diastolic dimension than control mice (4.3 +/- 0.2 vs. 3.7 +/- 0.1 mm; P < 0.05). Baseline heart rates were elevated in conscious Gs alpha mice (722 +/- 27 beats/min; n = 5) compared with control mice (656 +/- 28 beats/min; n = 5). Moreover, electrocardiographic monitoring demonstrated a high incidence of arrhythmias. Increased mortality compared with control mice (31.6 vs. 3.0%; P< 0.01) was also observed. Thus older mice with Gs alpha overexpression exhibit many of the features of dilated cardiomyopathy. This study supports the concept that chronic sympathetic stimulation over an extended period of time, i.e., over the life of an animal, is deleterious and actually may result in cardiomyopathy.

scholarly journals A case series on inflammatory cardiomyopathy and suspected cardiac sarcoidosis: role of cardiac PET in management

2020 ◽  
Vol 4 (4) ◽  
pp. 1-9
Author(s):  
Peter J Kennel ◽  
Farhan Raza ◽  
Jiwon Kim ◽  
Parmanand Singh ◽  
Alain Borczuk ◽  
...  
Keyword(s):  
Cardiac Sarcoidosis ◽  
Significant Coronary Artery Disease ◽  
Left Ventricular ◽  
Pet Scan ◽  
Left Ventricular Ejection ◽  
Cardiomyocyte Hypertrophy ◽  
Inflammatory Cardiomyopathy ◽  
Ventricular Ejection Fraction ◽  
New Onset

Abstract Background Presentation of life-threatening arrhythmias concomitantly with a new-onset non-ischaemic cardiomyopathy raises concern for an inflammatory cardiomyopathy such as cardiac sarcoidosis or cardiac manifestations of connective tissue disease. Comprehensive workup for specific aetiologies may be unrevealing except for signs of myocardial inflammation identified on cardiac positron emission tomography (PET). Here, we present five cases of such subjects and their clinical course. Case summary We collected clinical, imaging, pathological, and follow-up data of five subjects presenting with arrhythmias and unexplained new-onset cardiomyopathy. Mean age was 56.2 ± 5.8 years. Three subjects presented with ventricular tachycardia and two with atrial arrhythmias. Echocardiography showed a mean left ventricular ejection fraction of 37 ± 9%. Significant coronary artery disease was ruled out in all cases as the cause of the cardiomyopathy. All patients underwent cardiac magnetic resonance imaging (MRI) and PET scan at presentation and follow-up. In all patients, cardiac MRI revealed hyperenhancement in epicardial and mid-myocardial pattern in a non-coronary distribution, while PET scan revealed fluorodeoxyglucose (FDG) mismatch defects in multiple foci in a non-coronary distribution. Right ventricular biopsy was obtained in all patients and revealed interstitial fibrosis and cardiomyocyte hypertrophy. On median follow-up of 210 days, all subjects had improvement in both heart failure symptoms and arrhythmias and repeat PET in four out of five patients showed decreased inflammation. Discussion A high level of suspicion for inflammatory cardiomyopathy is needed in patients presenting with new unexplained cardiomyopathy and arrhythmias. A cardiac FDG-PET should be considered for diagnosis if cardiac inflammation is in the differential. This can inform further decisions regarding targeted immunomodulation therapy that may be helpful in this cohort.

scholarly journals Parasympathetic effects on cardiac electrophysiology during exercise and recovery in patients with left ventricular dysfunction

2009 ◽  
Vol 297 (2) ◽  
pp. H743-H749 ◽  
Author(s):  
Alexandru B. Chicos ◽  
Prince J. Kannankeril ◽  
Alan H. Kadish ◽  
Jeffrey J. Goldberger
Keyword(s):  
Left Ventricular Ejection Fraction ◽  
Sudden Death ◽  
Ejection Fraction ◽  
Left Ventricular Dysfunction ◽  
Cardiac Electrophysiology ◽  
Cycle Length ◽  
Ventricular Dysfunction ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction

Depressed parasympathetic activity has been proposed to be associated with an increased risk of sudden death. Parasympathetic effects (PE) on cardiac electrophysiology during exercise and recovery have not been studied in patients with left ventricular dysfunction. We performed noninvasive electrophysiological studies (NI-EPS) and characterized the electrophysiological properties of the sinus node, atrioventricular (AV) node, and ventricle in subjects with depressed left ventricular ejection fraction and dual-chamber defibrillators. NI-EPS were performed during rest, exercise, and recovery at baseline and after parasympathetic blockade with atropine to assess PE (the difference between parameter values in the 2 conditions). Ten subjects (9 men: age, 60 ± 9 yr; and left ventricular ejection fraction, 29 ± 8%) completed the study. All NI-EPS parameters decreased during exercise and trended toward rest values during recovery. PE at rest, during exercise, and during recovery, respectively, were on sinus cycle length, 320 ± 71 ( P = 0.0001), 105 ± 60 ( P = 0.0003), and 155 ± 82 ms ( P = 0.0002); on AV block cycle length, 137 ± 136 ( P = 0.09), 37 ± 19 ( P = 0.002), and 61 ± 39 ms ( P = 0.006); on AV interval, 58 ± 32 ( P = 0.035), 22 ± 13 ( P = 0.002), and 36 ± 20 ms ( P = 0.001); on ventricular effective refractory period, 15.8 ± 11.3 ( P = 0.02), 4.7 ± 15.2 ( P = 0.38), and 6.8 ± 15.5 ms ( P = 0.20); and on QT interval, 13 ± 12 ( P = 0.13), 3 ± 17 ( P = 0.6), and 20 ± 23 ( P = 0.04). In conclusion, we describe for the first time the changes in cardiac electrophysiology and PE during rest, exercise, and recovery in subjects with left ventricular dysfunction. PEs are preserved in these patients. Thus the role of autonomic changes in the pathophysiology of sudden death requires further exploration.

scholarly journals Cardiovascular phenotyping of the first mouse model of Sarcoidosis

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
C Bueno Beti ◽  
C Lim ◽  
A Protonotarios ◽  
A Kiss ◽  
M.N Sheppard ◽  
...  
Keyword(s):  
Mouse Model ◽  
Molecular Mechanisms ◽  
Left Ventricular ◽  
Cell Junctions ◽  
Left Ventricular Ejection ◽  
Unknown Etiology ◽  
Funding Source ◽  
Ventricular Ejection Fraction ◽  
Arrhythmogenic Substrate ◽  
Inflammatory Infiltrates

Abstract Introduction Sarcoidosis is a potentially life-threatening, inflammatory, granulomatous disease that affects multiple organs including the heart. Heretofore, its unknown etiology had hindered the creation of experimental models and the understanding of the molecular mechanisms of pathogenesis behind it. Purpose To extensively phenotype the heart of the first mouse model of sarcoidosis created through deletion of the tuberous sclerosis 2 (Tsc2) gene in the CD11c-positive macrophage population. Methods Tsc2 fl/fl CD11c Cre+ (Tsc2-KO; n=7) and Tsc2 fl/fl CD11c Cre- (Tsc2-WT; n=7) mice were subjected to echocardiography at 25 weeks of age (woa) to assess myocardial dimensions and function. Hearts of 13 and 25woa animals were subjected to histological and immunological stains to assess tissue changes, subtype inflammatory infiltrates and examine the localization of key proteins shown to be re-distributed in patients. Results At 13 woa, Tsc2-KO animals show inflammatory infiltrates; subtyped mainly as macrophages as well as evidence of myocyte destruction. At 25 woa, the number of inflammatory cells is significantly higher and there is heavy fibrotic replacement primarily in the septum and trabeculae. Older animals also show giant cells and non-necrotizing granulomas. The hearts show heterogeneous gap junction remodeling known to constitute an arrhythmogenic substrate and lack of immunoreactive signal for the desmosomal protein plakoglobin from the cell-cell junctions just as described in patients. The left ventricular ejection fraction and LV morphology was not significantly different between the two groups (EF: 64±4% in Tsc2-KO vs 64±2% in Tsc2-WT; LV end-systolic diameter: 4.51±0.54 mm in Tsc2-KO vs 4.59±0.29 mm in Tsc2-WT). However, there was a strong trend towards increasing filling pressure (E/e'ratio; 14.24±4.01 vs 12.15±2.54) and mean pulmonary pressure (21±6 vs 18±3 mmHg) in Tsc2-KO mice compared to controls suggesting diastolic dysfunction. Conclusion Hearts of the Tsc2 fl/fl CD11c Cre+ animals show a phenotype highly reminiscent of cardiac sarcoidosis in patients. We anticipate that this model will be very useful in deciphering molecular mechanisms of pathogenesis as well as testing much-needed mechanism-based therapies. Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): British Heart Foundation - PG/18/27/33616

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