scholarly journals Case Report: TNFα Antagonists Are an Effective Therapy in Cardiac Sarcoidosis

2021 ◽  
Vol 8 ◽  
Author(s):  
Julien Stievenart ◽  
Guillaume Le Guenno ◽  
Marc Ruivard ◽  
Virginie Rieu ◽  
Marc André ◽  
...  
Keyword(s):  
Cardiac Sarcoidosis ◽  
Immunosuppressive Agents ◽  
Case Series ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Cardiac Events ◽  
Serious Adverse Events ◽  
Caucasian Men ◽  
Life Threatening

Introduction: Cardiac sarcoidosis (CS) is a life-threatening disease in which clear recommendations are lacking. We report a case series of CS successfully treated by tumor necrosis factor (TNF)α antagonists.Methods: We conducted a single-center retrospective study of our patients with CS treated by TNFα antagonists.Results: Four cases (4/84, 4.7%) were found in our database. Mean age was 40 years (range 34–53 years), and all were Caucasian men. Mean follow-up was 54.75 months (range 25–115 months). All patients received corticosteroid therapy (CT) and immunosuppressive therapy (IT). TNFα antagonists (infliximab or adalimumab) were started after the first or second CS relapse under CT and IT. One patient experienced relapse under TNFα antagonists (isolated decreased left ventricular ejection) and responded to a shorter interval of TNFα antagonist infusion. CT was discontinued in three patients treated with TNFα antagonists without relapse or major cardiac events during follow-up. No serious adverse event occurred in our case series, possibly due to dose sparing and frequent arrest of CT.Conclusion: TNFα antagonists were effective in refractory and/or relapsing CS treated by corticosteroids and/or immunosuppressive agents, without serious adverse events, and should be considered earlier in CS treatment scheme.

scholarly journals Cardiac sarcoidosis: systematic review of literature on corticosteroid and immunosuppressive therapies

2021 ◽  
pp. 2100449
Author(s):  
Julien Stievenart ◽  
Guillaume Le Guenno ◽  
Marc Ruivard ◽  
Virginie Rieu ◽  
Marc André ◽  
...  
Keyword(s):  
Adverse Events ◽  
Cardiac Sarcoidosis ◽  
Positive Impact ◽  
Immunosuppressive Agents ◽  
Left Ventricular ◽  
Randomised Control Trial ◽  
Cochrane Library ◽  
Prisma Statement ◽  
Threatening Condition ◽  
Life Threatening

BackgroundCardiac sarcoidosis (CS) is a life-threatening condition in which clear recommendations are lacking. We aimed to review systematically the literature on cardiac sarcoidosis treated by corticosteroids and/or immunosuppressive agents in order to update the management of CS.MethodsUsing Pubmed, Embase and Cochrane Library databases, we found original articles on corticosteroid and/or standard immunosuppressive therapies for CS which provided at least fair SIGN overall assessment of quality and analyse the relapse rate, major cardiac adverse events (MACEs) and adverse events. We base our methods on Prisma statement and checklist.ResultsWe retrieved 21 studies. Mean quality provided by SIGN assessment was 6.8/14 (range 5–9). Corticosteroids appeared to have a positive impact on left ventricular function, atrioventricular block, and ventricular arrhythmias. For corticosteroids alone, nine (45%) studies (n=351) provided data on relapses, representing an incidence of 34% (n=119). Three studies (14%, n=73) provided data on MACEs (n=33), representing 45% of MACEs in patients treated by corticosteroid alone. Nine studies provided data on adjunctive immunosuppressive therapy in which four studies (n=78) provided data on CS relapse, representing an incidence of 33% (n=26). Limitations consisted in no randomised control trial retrieved and unclear data on MACEs in patients treated by combined immunosuppressive agents and corticosteroids.ConclusionsCorticosteroids should be started early after diagnosis but the exact scheme is still unclear. Studies concerning adjunctive conventional immunosuppressive therapies are lacking and benefits of adjunctive immunosuppressive therapies are unclear. Homogenous data on CS long-term outcomes under corticosteroids, immunosuppressive therapies and other adjunctive therapies are lacking.

Predicting mortality for patients with heart failure beyond oxygen consumption: a prognostic risk score

2021 ◽  
Author(s):  
Hanaa Shafiek ◽  
Andres Grau ◽  
Jaume Pons ◽  
Pere Pericas ◽  
Xavier Rossello ◽  
...  
Keyword(s):  
Heart Failure ◽  
Chronic Heart Failure ◽  
Risk Score ◽  
Cardiac Transplantation ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Functional Evaluation ◽  
Cardiac Events ◽  
Ventricular Ejection Fraction

Background: Cardiopulmonary exercise test (CPET) is a crucial tool for the functional evaluation of cardiac patients. We hypothesized that VO2 max and VE/VCO2 slope are not the only parameters of CPET able to predict major cardiac events (mortality or cardiac transplantation urgently or elective). Objectives: We aimed to identify the best CPET predictors of major cardiac events in patients with severe chronic heart failure and to propose an integrated score that could be applied for their prognostic evaluation. Methods: We evaluated 140 patients with chronic heart failure who underwent CPET between 2011 and 2019. Major cardiac events were evaluated during follow-up. Univariate and multivariate logistic regression analysis were applied to study the predictive value of different clinical, echocardiographic and CPET parameters in relation to the major cardiac events. A score was generated and c-statistic was used for the comparisons. Results: Thirty-nine patients (27.9%) died or underwent cardiac transplantation over a median follow-up of 48 months. Five parameters (maximal workload, breathing reserve, left ventricular ejection fraction, diastolic dysfunction and non-idiopathic cardiomyopathy) were used to generate a risk score that had better risk discrimination than NYHA dyspnea scale, VO2 max, VE/VCO2 slope > 35 alone, and combined VO2 max and VE/VCO2 slope (p= 0.009, 0.004, < 0.001 and 0.005 respectively) in predicting major cardiac events. Conclusions: A composite score of CPET and clinical/echocardiographic data is more reliable than the single use of VO2max or combined with VE/VCO2 slope to predict major cardiac events.

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.

scholarly journals P1525 Residual functional mitral regurgitation post-MitraClip is associated with worse hemodynamics and predicts poor outcome at 2-year follow-up

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
C M Van De Heyning ◽  
P Debonnaire ◽  
P B Bertrand ◽  
P Mortelmans ◽  
S Deferm ◽  
...  
Keyword(s):  
Heart Failure ◽  
Mitral Regurgitation ◽  
Independent Predictor ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Functional Mitral Regurgitation ◽  
Cardiac Events ◽  
History Of ◽  
Exercise Hemodynamics

Abstract Background Percutaneous mitral valve repair using MitraClip offers symptomatic benefit and improves rest and exercise hemodynamics in patients with severe functional mitral regurgitation (MR). Recent randomized trials have shown contradictory results regarding the impact of MitraClip on mid-term survival in functional MR. It is unknown whether improved hemodynamics are related to patients" outcome. Purpose To assess whether residual MR and altered resting and exercise hemodynamics are predictors of outcome in patients with functional MR treated with MitraClip. Methods Consecutive patients (n = 45, 72 ± 10years, left ventricular ejection fraction (LVEF) 34 ± 9%) with symptomatic severe functional MR were prospectively evaluated by Doppler echocardiography at rest and during symptom-limited exercise on a semi-supine bicycle pre- and 6 months post-MitraClip procedure. LVEF, MR severity, cardiac output (CO), systolic pulmonary artery pressure (SPAP) and a flow-corrected SPAP/CO ratio were assessed at rest and peak exercise. 2-year follow-up clinical data were collected from patient records. Results During 2-year follow-up post-MitraClip, 15 patients (33%) experienced major cardiac events (hospitalization for heart failure (n = 14) and/or cardiac death (n = 5)). Age, gender, a history of coronary artery disease, diabetes, baseline MR severity and baseline SPAP/CO ratio at rest and during exercise were not related to a worse event-free survival. In contrast, patients with events at 2-year follow up had more often a history of hospitalization for heart failure (73 vs. 37%, p = 0.029), lower baseline LVEF (30 ± 8 vs. 36 ± 10%, p = 0.041), more residual MR at 6 months post-MitraClip (MR jet area/left atrial area 27 ± 14 vs. 15 ± 10%, p = 0.004) and higher SPAP/CO ratios at rest and during exercise 6 months post-MitraClip (13.9 ± 5.3 vs. 9.9 ± 3.4mmHg/L/min, p = 0.007 and 13.6 ± 4.9 vs. 9.4 ± 4.6mmHg/L/min, p = 0.009, respectively). When corrected for baseline LVEF, residual MR 6 months post-MitraClip remained an independent predictor for worse 2-year outcome. Residual MR was moderately correlated to a worse SPAP/CO ratio 6 months post-MitraClip (Pearson Rho 0.518, p &lt; 0.001). Conclusions In patients with functional MR treated with MitraClip, residual MR at 6-month follow-up is associated with impaired hemodynamics, and is an independent predictor of cardiac events at 2-year follow-up.

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.

Can peripartum cardiomyopathy be caused by chemotherapy and radiation of breast cancer?

2013 ◽  
Vol 2 (1-2) ◽  
Author(s):  
Andreas Kyvernitakis ◽  
Ioannis Kyvernitakis ◽  
Alexander Yang ◽  
Ute-Susann Albert ◽  
Stephan Schmidt ◽  
...  
Keyword(s):  
Primary Treatment ◽  
Left Breast ◽  
Left Ventricular ◽  
Peripartum Cardiomyopathy ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Surgical Interventions ◽  
Life Threatening ◽  
Cardiac Intensive Care

AbstractTo report on a pregnant woman with peripartum cardiomyopathy 7 years after combination chemotherapy with doxorubicine and radiation of cancer of the left breast.A 35-year old primigravida who was treated 7 years earlier with cancer of the left breast (ympT1c, ypN0, cM0), according to a neoadjuvant study protocol (GeparTrio), was transferred to our unit due to HELLP syndome at 35+5 weeks. Symptoms of cardiopulmonary decompensation occurred shortly after cesarean delivery of a healthy newborn. The patient was admitted to cardiac intensive care and treated with oxygen, diuretics and ACE inhibitors. Maternal left ventricular ejection fraction recovered within a few weeks without any surgical interventions and remained stable within 1 year of follow-up.The association between radical primary treatment of the left breast and life-threatening cardiac disease could possibly be provoked by pregnancy.

Trastuzumab-Associated Cardiac Events at 8 Years of Median Follow-Up in the Herceptin Adjuvant Trial (BIG 1-01)

2014 ◽  
Vol 32 (20) ◽  
pp. 2159-2165 ◽  
Author(s):  
Evandro de Azambuja ◽  
Marion J. Procter ◽  
Dirk J. van Veldhuisen ◽  
Dominique Agbor-Tarh ◽  
Otto Metzger-Filho ◽  
...  
Keyword(s):  
Early Stage ◽  
Growth Factor Receptor ◽  
Severe Congestive Heart Failure ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Cardiac Events ◽  
Her2 Positive ◽  
Ventricular Ejection Fraction ◽  
Low Incidence

Purpose To document the rate and outcome of trastuzumab-associated cardiac dysfunction in patients following 1 or 2 years of adjuvant therapy. Patients and Methods The Herceptin Adjuvant (HERA) trial is a three-arm, randomized trial comparing 2 years or 1 year of trastuzumab with observation in 5,102 patients with human epidermal growth factor receptor 2 (HER2) –positive early-stage breast cancer. Cardiac function was closely monitored. Eligible patients had left ventricular ejection fraction (LVEF) ≥ 55% at study entry following neoadjuvant chemotherapy with or without radiotherapy. This 8-year median follow-up analysis considered patients randomly assigned to 2 years or 1 year of trastuzumab or observation. Results The as-treated safety population for 2 years of trastuzumab (n = 1,673), 1 year of trastuzumab (n = 1,682), and observation (n = 1,744) is reported. Cardiac adverse events leading to discontinuation of trastuzumab occurred in 9.4% of patients in the 2-year arm and 5.2% of patients in the 1-year arm. Cardiac death, severe congestive heart failure (CHF), and confirmed significant LVEF decrease remained low in all three arms. The incidence of severe CHF (0.8%, 0.8%, and 0.0%, respectively) and confirmed significant LVEF decrease (7.2%, 4.1%, and 0.9%, respectively) was significantly higher in the 2-year and 1-year trastuzumab arms compared with the observation arm. Severe CHF was the same for 2-year and 1-year trastuzumab. Of patients with confirmed LVEF decrease receiving 2-year trastuzumab, 87.5% reached acute recovery. Of patients with confirmed LVEF decrease receiving 1-year trastuzumab, 81.2% reached acute recovery. Conclusion Long-term assessment at 8-year median follow-up confirms the low incidence of cardiac events for trastuzumab given sequentially after chemotherapy and radiotherapy, and cardiac events were reversible in the vast majority of patients.

scholarly journals Long time clinical course of cardiac sarcoidosis with corticosteroid therapy -from Japanese nationwide questionnaire survey-

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
H Kamada ◽  
K Ishibashi ◽  
K Nakajima ◽  
N Ueda ◽  
T Kamakura ◽  
...  
Keyword(s):  
Ventricular Arrhythmia ◽  
Corticosteroid Therapy ◽  
Questionnaire Survey ◽  
Clinical Characteristics ◽  
Cardiac Involvement ◽  
Cardiac Sarcoidosis ◽  
Japanese Patients ◽  
Left Ventricular ◽  
Left Ventricular Ejection

Abstract Background Sarcoidosis is a systemic inflammatory syndrome of unknown etiology and cardiac involvement has been reported to be an important prognostic factor in this disease. An autopsy study has reported that the frequency of this cardiac involvement (cardiac sarcoidosis: CS) varies in the different countries and races and very frequent in Japanese patients. We therefore performed the nationwide questionnaire survey and try to clarify the clinical characteristics and corticosteroid effect in CS, especially focused on arrhythmic events in this disease. Methods Total of 757 Japanese patients from 57 hospitals who diagnosed CS were examined. Patients who unsatisfied the criteria of the Japanese new guidelines, or who underwent cardiac transplantations were excluded, and 420 patients (287 females, median follow-up periods 1864 days [interquartile range: 845–3159 days]) were analyzed. The clinical outcome and corticosteroid effect were evaluated. Results Clinical characteristics at diagnosis was as follows: female dominant (68%), mean age of 60±13 years old, mean left ventricular ejection fraction was 49±16%. Arrhythmic events were very frequently observed as an initial cardiac manifestation in 263 patients (62%) of CS, of which atrioventricular block (AVB) in 174 (41%), ventricular tachycardia (VT) in 73 (17%) and AVB with VT in 17 (4%) (Figure 1A). Pacemaker was implanted in 166 patients (40%) and defibrillators was 137 patients (33%). Corticosteroid was prescribed in 144 (83%) of 174 patients with AVB and in 62 (85%) of 73 patients with VT. Initial dose was mean 47.9 mg and maintenance dose of mean 7.3 mg. Corticosteroid improved VT as good as AVB (27% vs. 29%). However, corticosteroid sometimes worsened VT events compared with AVB (10% vs. 2%) (Figure 1B). During the course of follow-up, 32 patients were needed to increase corticosteroid in 23 of AVB and 10 of VT cases. However, there were no difference in mortality between the groups, whether or not to increase corticosteroid. All survival rate was 92% (5-year mortality), 83% (10-year mortality) and free from all cause death and defibrillator charge was 81% (5 year), 71% (10 year). Conclusion Fatal arrhythmia is commonly observed in CS as a primary symptom. Corticosteroid sometimes worsen ventricular arrhythmia and appropriate defibrillator discharge was common. Thus, careful attention for activating ventricular arrhythmia would be needed during the follow-up period even after corticosteroid therapy. Funding Acknowledgement Type of funding source: None

Prognostic implications of late gadolinium enhancement for re-worsening left ventricular ejection fraction in patients with dilated cardiomyopathy: a longitudinal study of left ventricular function

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Nabeta ◽  
S Ishii ◽  
Y Ikeda ◽  
K Maemura ◽  
T Oki ◽  
...  
Keyword(s):  
Multivariate Logistic Regression Analysis ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Multivariate Logistic Regression ◽  
Cardiac Events ◽  
Gadolinium Enhancement ◽  
Ventricular Ejection Fraction ◽  
Recent Onset ◽  
Lge Cmr

Abstract Background Re-worsening left ventricular ejection fraction (LVEF) after initial recovery occurs in some patients with dilated cardiomyopathy (DCM). However, prevalence and predictors of re-worsening LVEF in longitudinal follow-up are unclear. Late gadolinium enhancement of cardiovascular magnetic resonance (LGE-CMR) can evaluate the damage of myocardial tissue. Purpose This study sought to evaluate the clinical parameters including LGE-CMR to predict re-worsening LVEF in patients with recent-onset DCM. Methods We included patients with recent-onset DCM who had an LVEF &lt;45% and underwent LGE-CMR at diagnosis. We performed yearly echocardiographc follow-up [median 6 [4–8.3] years]. Initial LVEF recovery defined as patients increased in &gt;5% LVEF from baseline and had an LVEF≥45% after medical therapy. Patients were divided into three groups: (1) Improved: defined as those with sustained LVEF ≥45% after initial LVEF recovery; (2) Re-worse: those with decreased &gt;5% and had an LVEF &lt;45% after initial LVEF recovery. and (3) Not-improved: those with no initial LVEF recovery during follow-up. To evaluate the prognostic factors for Re-worsening LVEF after initial LVEF recovery, multivariate logistic regression analysis performed between the Improved group and the Re-worse group. Cardiac events defined as hospitalization due to heart failure and sudden death. Results Of 138 patents, 82 patients (59%) were the Improved group, 42 patients (30%) were the Re-worse group, and 14 (10%) were the Not-improved group. Loess curves of long-term LVEF trajectories showed that LVEF in the Re-worse group increased first 2 years and declined slowly thereafter (Fig. 1A). Re-worsening LVEF occurred 4.5±2.2 years after initial LVEF recovery. Multivariate logistic regression analysis demonstrated that LGE area at baseline (Odds ratio: 1.09, 95% confidence interval (CI) 1.02–1.18, p=0.014) and Log brain natriuretic peptide (BNP) at initial LVEF recovery (Odds ratio: 1.53, 95% confidence interval (CI) 1.01–2.31, p=0.042) were independent predictors for Re-worsening LVEF. Kaplan Meier analysis demonstrated that the risk of cardiac events in the Re-worse group was significantly higher (hazard ratio: 3.93, 95% CI 1.49–10.36, p=0.006) than in the Improved group and lower risk than in the Not-improved group (hazard ratio: 0.28, 95% CI 0.12–0.62, p=0.002) (Fig. 1B). Conclusion Re-worsening LVEF occurred in 30% of patients in patients with recent-onset DCM. LGE area and BNP at initial LVEF recovery were independently associated with re-worsening LVEF after initial LVEF recovery. Figure 1 Funding Acknowledgement Type of funding source: None

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