A Case of Cardiac Sarcoidosis Presenting Right-Sided Dominant Heart Failure with Low Output Syndrome

Objective: Although several studies have described the effects of adriamycin on the heart, electrocardiographic total 12-lead QRS voltage (distance in millimeters from the peak of the R wave to the nadir of either the Q or S wave, whichever was deeper, with 10 mm [1 mV] being standard) both before and after orthotopic heart transplantation (OHT) has not been reported. This study describes the total 12-lead QRS voltage in 8 patients studied at Baylor University Medical Center at Dallas, from 1994 to June 2018, who underwent OHT for severe heart failure caused by anthracycline-induced cardiomyopathy. Method: Prior to OHT, the total 12-lead non-paced QRS voltages ranged from 86 to 189 mm (mean 125 ± 56) and for paced QRS voltages from 82 to 113 mm (mean 97 ± 15). The total 12-lead QRS voltages post-OHT ranged from 100 to 190 mm (mean 130 ± 30). Total 12-lead QRS voltages were lower in patients with a pacemaker than without. Results/Conclusion: These low voltages are like those found in patients with carcinoid syndrome, severe cardiac adiposity, cardiac amyloidosis, and cardiac sarcoidosis.

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Clinical phenotypes of cardiac sarcoidosis by latent class analysis

European Heart Journal ◽

10.1093/eurheartj/ehab724.2763 ◽

2021 ◽

Vol 42 (Supplement_1) ◽

Author(s):

Q Dai ◽

B Bose ◽

P Li ◽

B Liu ◽

L Jin ◽

...

Keyword(s):

Heart Failure ◽

Latent Class Analysis ◽

Clinical Outcomes ◽

Ventricular Arrhythmias ◽

Latent Class ◽

Cardiac Sarcoidosis ◽

Information Criterion ◽

Cardiometabolic Syndrome ◽

Study Population ◽

Myocardial Involvement

Abstract Background Sarcoidosis is a systemic granulomatous disease with cardiac involvement reported in 20–27% of patients [1]. Cardiac sarcoidosis (CS) can lead to atrial or ventricular arrhythmias, various conduction system disorders, heart failure or sudden cardiac death, depending on the location of myocardial involvement [2]. Previous studies have investigated the possible types of CS based on the distribution of myocardial involvement on imaging as well as the role of genetic factors [3,4]. However, there are no studies describing the clinical heterogeneity of CS patients. Purpose In order to determine if clinical clusters exist in CS, we carried out a latent class analysis (LCA) to explore potential phenotypes in a large sample of CS patients from the National Inpatient Sample (NIS). Methods We identified 848 patients with a diagnosis of CS from the NIS in 2016–2018. A LCA was performed based on comorbidities. Utilizing the Bayesian information criterion and Akaike's information criterion we divided our study population into 3 cohorts. We subsequently applied the LCA model for our study population to fit each patient into one of the 3 cohorts. Finally, we compared the clinical outcomes among the 3 groups. Results Following LCA, patients in cohort 3 were strongly associated with a cardiometabolic syndrome profile with the highest prevalence of congestive heart failure (CHF, 95.1%), chronic kidney disease (CKD, 69.7%), diabetes mellitus (68.9%), hyperlipidemia (52.5%) and obesity (45.1%). Patients in cohort 2 had an intermediate prevalence of cardiometabolic syndrome with a universal diagnosis of hypertension (100%) but with the lowest number of CHF (32.5%) patients and none with CKD. Finally, patients in cohort 1 had the least comorbidities in comparison to the other groups but there was a higher prevalence of CHF (71.7%). There was no significant difference in mortality among the 3 groups, but acute respiratory failure was the highest in cohort 3. However, ventricular arrhythmias were more prevalent in cohort 1 patients (Table). Conclusion We identified 3 different types of CS based on their clinical phenotype. The clinical outcomes varied among the cohorts with ventricular arrhythmias being the most prevalent in patients with the least cardiometabolic comorbidities. FUNDunding Acknowledgement Type of funding sources: None.

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The prevalence of cardiac sarcoidosis is underestimated: [18F]FDG-PET is the answer

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeab111.071 ◽

2021 ◽

Vol 22 (Supplement_3) ◽

Author(s):

J Borges-Rosa ◽

M Oliveira-Santos ◽

R Silva ◽

J Lopes De Almeida ◽

L Goncalves ◽

...

Keyword(s):

Heart Failure ◽

Fdg Pet ◽

Cardiac Involvement ◽

Cardiac Sarcoidosis ◽

Clinical Manifestations ◽

European Population ◽

Response To Therapy ◽

Histological Diagnosis ◽

18F Fdg

Abstract Funding Acknowledgements Type of funding sources: None. Background Overt cardiac involvement is reported in 5% of patients with sarcoidosis, although autopsy and imaging studies suggest higher prevalence, worldwide variation. The role of 18F-fluorodeoxyglucose positron emission tomography ([18F]FDG-PET) in non-invasive diagnosis and follow-up has increased in the last decade. Purpose Our goal is to describe the prevalence, clinical manifestations and outcomes of cardiac sarcoidosis (CS), diagnosed through [18F]FDG-PET, in a southern European population. Methods We included all patients with histological diagnosis of extracardiac sarcoidosis screened with [18F]FDG-PET between 2009 and 2020. We collected data on clinical manifestations, cardiac magnetic resonance (CMR) results, and mortality outcomes and compared those with and without cardiac involvement. We applied the criteria for the diagnosis of CS from Heart Rhythm Society. Results Of the 400 patients screened with [18F]FDG-PET, 128 had a histological diagnosis of extracardiac sarcoidosis (54.7% females, mean age 51.0 ± 14.2 years). None underwent endomyocardial biopsy. Ten patients had a pattern of [18F]FDG uptake consistent with CS defined as diffuse (n = 5), focal (n = 3), and focal on diffuse (n = 2). Of the 128 patients, 14 also underwent CMR, which identified 2 subjects with positive findings in both modalities and 3 additional patients: focal (n = 1), multifocal mid-wall (n = 2), focal mid-wall (n = 2), and multifocal subepicardial (n = 1) delayed gadolinium enhancement. Overall, 13 patients (10.2%) fulfilled the criteria for probable CS (53.8% female, mean age 56.2 ± 12.6 years), all with multiorgan involvement, mostly lung and lymph nodes (each 92%), followed by skin and central nervous system (each 15%). Median left ventricle ejection fraction was 62% [55-65] and there were cardiac manifestations of CS in 6 patients (46%): sick sinus syndrome (n = 2), complete heart block (n = 1), frequent premature ventricular complexes (n = 1), ventricular tachycardia plus heart failure (n = 1), and bifascicular block plus heart failure (n = 1). Eleven patients (85%) with probable CS were medicated with immunosuppressant drugs: corticosteroids (n = 9), methotrexate (n = 4), and azathioprine (n = 2). Four patients with previous [18F]FDG screening were revaluated after treatment, each showing no cardiac uptake. After a mean follow-up of 4.0 ± 1.0 years, mortality was three-fold higher in patients with cardiac involvement, despite the absence of statistical significance (15% vs. 5%, P = 0.151). Conclusions In a southern European population with histological extracardiac sarcoidosis, the prevalence of cardiac involvement was 10.2%, most asymptomatic. [18F]FDG-PET improves the diagnostic yield and plays an important role in monitoring response to therapy. The higher mortality trend in those with CS needs to be ascertained in longer follow-up.

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

Circulation ◽

10.1161/circ.118.suppl_18.s_1033-b ◽

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.

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Cardiac Sarcoidosis

Seminars in Respiratory and Critical Care Medicine ◽

10.1055/s-0040-1712535 ◽

2020 ◽

Vol 41 (05) ◽

pp. 626-640 ◽

Cited By ~ 1

Author(s):

David H. Birnie

Keyword(s):

Heart Failure ◽

Cardiac Involvement ◽

Cardiac Sarcoidosis ◽

Positron Emission Tomography Imaging ◽

Left Ventricular ◽

Implantable Cardioverter Defibrillators ◽

Heart Failure Treatment ◽

Positron Emission ◽

Minimal Data ◽

Cardioverter Defibrillators

AbstractApproximately 5% of patients with sarcoidosis will have clinically manifest cardiac involvement presenting with one or more of ventricular arrhythmias, conduction abnormalities, and heart failure. It is estimated that another 20 to 25% of pulmonary/systemic sarcoidosis patients have asymptomatic cardiac involvement (clinically silent disease). Cardiac presentations can be the first (and/or an unrecognized) manifestation of sarcoidosis in a variety of circumstances. Immunosuppression therapy (usually with corticosteroids) has been suggested for the treatment of clinically manifest cardiac sarcoidosis (CS) despite minimal data supporting it. Positron emission tomography imaging is often used to detect active disease and guide immunosuppression. Patients with clinically manifest disease often need device therapy, typically with implantable cardioverter defibrillators (ICDs). The extent of left ventricular dysfunction seems to be the most important predictor of prognosis among patients with clinically manifest CS. In the current era of earlier diagnosis, modern heart failure treatment, and use of ICD therapy, the prognosis from CS is much improved. In a recent Finnish nationwide study, 10-year cardiac survival was 92.5% in 102 patients.

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Clinical Features and Histopathology of Cardiac Sarcoidosis with Refractory Heart Failure: An Autopsy Case

Internal Medicine ◽

10.2169/internalmedicine.3147-19 ◽

2019 ◽

Vol 58 (24) ◽

pp. 3551-3555

Author(s):

Fumio Terasaki ◽

Hiroko Kuwabara ◽

Yoshihiro Takeda ◽

Yohei Yamauchi ◽

Shuichi Fujita ◽

...

Keyword(s):

Heart Failure ◽

Clinical Features ◽

Cardiac Sarcoidosis ◽

Autopsy Case ◽

Refractory Heart Failure

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Heart Transplantation for End-Stage Heart Failure Due to Cardiac Sarcoidosis

Transplantation Proceedings ◽

10.1016/j.transproceed.2013.02.116 ◽

2013 ◽

Vol 45 (6) ◽

pp. 2384-2386 ◽

Cited By ~ 31

Author(s):

D. Perkel ◽

L.S.C. Czer ◽

R.P. Morrissey ◽

A. Ruzza ◽

M. Rafiei ◽

...

Keyword(s):

Heart Failure ◽

Heart Transplantation ◽

Cardiac Sarcoidosis ◽

End Stage Heart Failure ◽

End Stage

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Chronic Heart Failure with Reduced Ejection Fraction Due to Cardiac Sarcoidosis Complicated Coronary Vasospastic Angina

Journal of Cardiac Failure ◽

10.1016/j.cardfail.2015.08.112 ◽

2015 ◽

Vol 21 (10) ◽

pp. S163

Author(s):

Shingo Yoshioka ◽

Takashi Shimozato ◽

Hirotaka Ohtake ◽

Masayuki Nakamura ◽

Akimitsu Tanaka ◽

...

Keyword(s):

Heart Failure ◽

Chronic Heart Failure ◽

Ejection Fraction ◽

Cardiac Sarcoidosis ◽

Vasospastic Angina ◽

Reduced Ejection Fraction

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834-6 Identification of cardiac sarcoidosis from the patients presenting as unexplained heart failure and cardiomyopathies

Journal of the American College of Cardiology ◽

10.1016/s0735-1097(04)90873-x ◽

2004 ◽

Vol 43 (5) ◽

pp. A205

Author(s):

Osamu Yokoseki ◽

Yoshikazu Yazaki ◽

Kaoru Kogashi ◽

Takeshi Hanaoka ◽

Yuichi Kai ◽

...

Keyword(s):

Heart Failure ◽

Cardiac Sarcoidosis

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Diagnostic and Predictive Value of Speckle Tracking Echocardiography in Cardiac Sarcoidosis

10.21203/rs.2.14614/v3 ◽

2019 ◽

Author(s):

Cristina Di Stefano ◽

Giulia Bruno ◽

Maria Arciniegas Calle ◽

Gayatri A. Acharya ◽

Lynn M. Fussner ◽

...

Keyword(s):

Heart Failure ◽

Speckle Tracking ◽

Cardiac Sarcoidosis ◽

Speckle Tracking Echocardiography ◽

Early Stage ◽

Systolic Function ◽

Left Ventricular ◽

Left Ventricular Ejection ◽

Ventricular Ejection Fraction ◽

Strain And Strain Rate

Abstract Background: Sarcoidosis is a systemic granulomatous disease that may affect the myocardium. This study evaluated the diagnostic and prognostic value of 2-dimensional speckle tracking echocardiography in cardiac sarcoidosis (CS). Methods: Eighty-three patients with extracardiac, biopsy-proven sarcoidosis and definite/probable diagnosis of cardiac involvement diagnosed from January 2005 through December 2016 were included. Strain parameters in early stages of CS, in a subgroup of 23 CS patients with left ventricular ejection fraction (LVEF) within normal limits (LVEF>52% for men: >54% for women, mean value: 57.3%±3.8%) and no wall motion abnormalities was compared with 97 controls (1:4) without cardiac disease. LV and right ventricular (RV) global longitudinal (GLS), circumferential (GCS), and radial (GRS) strain and strain rate (SR) analyses were performed with TomTec software and correlated with cardiac outcomes (including heart failure and arrhythmias). This study was approved by the Mayo Clinic Institutional Review Board, and all patients gave informed written consent to participate. Results: Mean age of CS patients was 53.6±10.8 years, and 34.9% were women. Mean LVEF was 43.2%±12.4%; LV GLS, -12.4%±3.7%; LV GCS, -17.1%±6.5%; LV GRS, 29.3%±12.8%; and RV wall GLS, 14.6%±6.3%. In the 23 patients with early stage CS with normal LVEF and RV systolic function, strain parameters were significantly reduced when compared with controls (respectively: LV GLS, -15.9%±2.5% vs -18.2%±2.7% [ P =.001]; RV GLS, -16.9%±4.5% vs -24.1%±4.0% [ P <.001]). A LV GLS value of -16.3% provided 82.2% sensitivity and 81.2% specificity for the diagnosis of CS (AUC 0.91), while a RV value of -19.9% provided 88.1% sensitivity and 86.7% specificity (AUC 0.93). Hospital admission and heart failure significantly correlated to impaired LV GLS (> -14%). Conclusion: Reduced strain values in the LV GLS and RV GLS can be used in the diagnostic algorithm in patients with suspicion of cardiac sarcoidosis. These values also correlate with adverse cardiovascular events.

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