scholarly journals Ventricular tachycardia in cardiac sarcoidosis -prognosis, characterization of ventricular substrates and outcomes of treatment-

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
R Tonegawa ◽  
K Miyamoto ◽  
N Ueda ◽  
K Nakajima ◽  
M Wada ◽  
...  

Abstract Background The prognosis, the underlying substrate and clinical outcomes of treatment are unclear in patients with cardiac sarcoidosis (CS)-related ventricular tachycardia (VT). Objective This study investigated the prognosis and the relationship between electroanatomical mapping (EAM) and imaging findings in patients with CS-related VT. Methods A total of 203 CS patients (Age 68.1±11.6 years, 87 males) were enrolled at two tertiary care medical centers between 2000 and 2018. All met the 2016 Japanese Circulation Society guidelines for diagnosis of CS. They were followed for a composite of major adverse cardiac events (MACE) including cardiac death, heart transplantation, unscheduled hospitalization for heart failure, and life-threatening ventricular arrhythmias. Distribution of late gadolinium enhancement (LGE) on cardiac MRI (CMR) and/or an abnormal myocardial 18F-fluorodeoxyglucose (FDG) uptake on positron emission tomography at diagnosis were examined. The relationship between EAM and the image findings were also analyzed in patients with radiofrequency ablation (RFA) for VT. Results During a median follow-up of 53 months, 87 of the 203 patients (43%) experienced a MACE. Baseline factors associated with MACE were presence of sustained VT (HR, 2.43, 95% CI 1.54–3.85, P<0.001), left ventricular ejection fraction below 50% (HR, 1.95 95% CI 1.07–3.56, P=0.029), and abnormal myocardial FDG uptake (HR, 2.42 95% CI 1.04–5.61, P=0.039). Overall, 69 of the 203 patients (34%) experienced sustained VT. Abnormal myocardial FDG uptake was significantly more prevalent in patients with VT than in those without (92.7% vs. 78.5%, P=0.02). A total of 25 patients (9.9%) required RFA for CS-related VT (Age 64.0±8.7 years, 12 males, 1.32±0.56 RFAs per patient). Abnormal electrocardiograms (EGM) were observed in 22 of the 25 patients (88%). LGE was more frequent than abnormal FDG uptake in areas with an abnormal EGM (77% vs. 41%; P=0.002). Over a mean follow-up period of 67-months, 13 of the 25 patients with RFA (52%) remained free of VT episodes (Figure). VT recurred in nine of the 12 patients with RFA and in 17 of the 47 patients without RFA, but was suppressed by intensive pharmacologic therapy such as the combined use of amiodarone and sotalol. In patients with CS-related VT, survival without experiencing a MACE did not differ in participants with or without RFA. Conclusions In our 203 CS patients, sustained VT and abnormal FDG uptake were associated with worse cardiac outcomes. The prevalence of abnormal FDG uptake was significantly higher in patients with CS-related VT, LGE on CMR was more frequent within localized areas of an abnormal EGM, suggesting that both scar itself and the associated inflammation were involved in the pathogenesis of CS-related VT. Successful RFA of CS-related VT is still challenging, and recurrence is common. Preprocedural CMR can be useful in detecting abnormal EGMs that are potential targets for substrate ablation. Funding Acknowledgement Type of funding source: None

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Yoshikazu Yazaki ◽  
Mitsuaki Horigome ◽  
Kazunori Aizawa ◽  
Takeshi Tomita ◽  
Hiroki Kasai ◽  
...  

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.


2020 ◽  
Vol 1 (1) ◽  
pp. 12-17
Author(s):  
Mehmet Küçükosmanoğlu ◽  
Cihan Örem

Introduction: MPI is an echocardiographic parameter that exibit the left ventricular functions globally. NT-proBNP  is an important both diagnostic and prognostic factor in heart failure. In this study, we aimed to investigate the prognostic significance of serum NT-proBNP levels and MPI in patients with STEMI. Method: Totally 104 patients with a diagnosis of STEMI were included in the study. Patients followed for 30-days and questioned for presence of symptoms of heart failure (HF) and cardiac death. Patients were invited for outpatient control after 30-days and were divided into two groups: (HF (+) group) and (HF (-) group). Results: Totally 104 patients with STEMI were hospitalized in the coronary intensive care unit. Of those patients, 17 were female (16%), 87 were male (84%), and the mean age of the patients was 58.9±10.8 years. During the 30-day follow-up, 28 (27%) of 104 patients developed HF. The mean age, hypertension ratio and anterior STEMI rate were significantly higher in the HF (+) group compared to the HF (-) group. Ejection time (ET) and left ventricular ejection fraction (LVEF) were significantly lower and MPI was significantly higher in the HF (+) group. When the values on day first and  sixth were compared, NT-ProBNP levels were decreased in both groups. There was no significant difference between the two groups in terms of the change in MPI values on the first and sixth days. Multiple regression analysis showed that the presence of anterior MI, first day NT-proBNP level and LVEF were independently associated with development of HF and death. Conclusion: In our study, NT-proBNP levels were found to be positively associated with MPI in patients with acute STEMI. It was concluded that the level of NT-proBNP detected especially on the 1st day was more valuable than MPI in determining HF development and prognosis after STEMI.  


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
P Simonen ◽  
J Lehtonen ◽  
M Kupari

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.


2020 ◽  
Vol 4 (4) ◽  
pp. 1-9
Author(s):  
Peter J Kennel ◽  
Farhan Raza ◽  
Jiwon Kim ◽  
Parmanand Singh ◽  
Alain Borczuk ◽  
...  

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.


PPAR Research ◽  
2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Izabela Wojtkowska ◽  
Tomasz A. Bonda ◽  
Jadwiga Wolszakiewicz ◽  
Jerzy Osak ◽  
Andrzej Tysarowski ◽  
...  

Activation of PPARs may be involved in the development of heart failure (HF). We evaluated the relationship between expression of PPARγin the myocardium during coronary artery bypass grafting (CABG) and exercise tolerance initially and during follow-up. 6-minute walking test was performed before CABG, after 1, 12, 24 months. Patients were divided into two groups (HF and non-HF) based on left ventricular ejection fraction and plasma proBNP level. After CABG, 67% of patients developed HF. The mean distance 1 month after CABG in HF was397±85 m versus420±93 m in non-HF. PPARγmRNA expression was similar in both HF and non-HF groups. 6MWT distance 1 month after CABG was inversely correlated with PPARγlevel only in HF group. Higher PPARγexpression was related to smaller LVEF change between 1 month and 1 year (R=0.18,p<0.05), especially in patients with HF. Higher initial levels of IL-6 in HF patients were correlated with longer distance in 6MWT one month after surgery and lower PPARγexpression. PPARγexpression is not related to LVEF before CABG and higher PPARγexpression in the myocardium of patients who are developing HF following CABG may have some protecting effect.


2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Vincenzo Nuzzi ◽  
Anne Raafs ◽  
Paolo Manca ◽  
Michiel T.h.M. Henkens ◽  
Caterina Gregorio ◽  
...  

Abstract Aims Left atrial (LA) dilation is associated a with worse prognosis in several cardiovascular settings but therapies can promote LA reverse remodelling. Characterizing and defining the prognostic implications of LA volume (LAVI) reduction in dilated cardiomyopathy (DCM). Methods and results Consecutive DCM patients from two tertiary care centres, with available echocardiography at baseline and at 1 year follow-up, were analysed. LA dilation was defined as LAVI &gt;34 ml/m2, Delta (Δ)LAVI was defined as the 1 year relative LAVI reduction. The outcome was a composite of death/heart transplantation/heart failure hospitalization (D/HTx/HFH). Five hundred sixty patients were included [age 52 ± 13 years; left ventricular ejection fraction (LVEF) 31 ± 10%, LAVI 45 ± 18 ml/m2]. Baseline LAVI had a non-linear association with the risk of D/HTx/HFH, independently from LVEF (P &lt; 0.001). At 1 year follow-up, LAVI decreased in 374 patients (67%, median ΔLAVI 24%, interquartile range 37% 11%). Factors independently associated with ΔLAVI were higher baseline LAVI and lower baseline LVEF. After adjustment for confounders, ΔLAVI showed a linear association with the risk of D/HTx/HHF (HR: 0.92, 95% CI: 0.86 0.99 per 5% decrease, P &lt; 0.001). At 1 year Follow-up, patients with a ≥ 15% reduction in ΔLAVI or LAVI normalization (i.e. Follow-up LAVI ≤34ml/m2) (42% of the cohort) were at lower risk of D/HTx/HFH (HR: 0.49, 95% CI: 0.33 0.74, P &lt; 0.001). Conclusions In a large cohort of DCM, 1 year reduction in LAVI is observed in the majority of patients. The association between reduction in LAVI and D/HTx/HHF candidates LA reverse remodelling as complementary early therapeutic goal in DCM.


2020 ◽  
Author(s):  
Joseph Odunga Abuodha ◽  
Asim Jamal Shaikh ◽  
Jasmit Shah ◽  
Mohamed Jeilan ◽  
Anders Barasa

Abstract Background Anthracyclines are associated with irreversible cardiotoxicity, with changes in echocardiographic parameters preceding clinically manifest cardiac dysfunction. We sought to evaluate the incidence of early cardiac dysfunction post anthracyclines, and associated clinical, echocardiographic and treatment parameters in a sub-Saharan African population. Methods Cancer patients aged ≥18years at anthracycline initiation with archived baseline echocardiograms, underwent repeat echocardiographic assessment. Cases (with cardiac dysfunction) had (1) >15% relative decline from baseline in global longitudinal strain (GLS), or (2) a decline in left ventricular ejection fraction (LVEF) from baseline to <53% with either (i) symptoms (assessed by the Duke Activity Status Index at follow-up echocardiogram) and LVEF decline by >5 to ≤10%, or (ii) LVEF decline >10% regardless of symptoms. Comparisons in clinical, echocardiographic and treatment parameters were made with controls (no cardiac dysfunction). Results Among 141 patients (mean age, 47.7years ± 11.2, Africans 95%, females 85.1%, breast cancer 82%), 39 (27.7%) had cardiac dysfunciton at a mean inter-echocardiogram interval of 14.9months ± 14.3, mean cumulative anthracycline dose of 244.7mg/m 2 ± 72.2, and mean DASI score was 50.0 ± 13.3. Mean cardiotoxic doxorubicin equivalence dose was 236.7mg/m 2 ± 57.4 for cases and 217.3 ± 61.9 for controls [p = 0.033, OR = 1.00 (95% CI: 0.99 - 1.01)]. The assessed clinical, echocardiographic and treatment parameters were not associated with cardiac dysfunction. Conclusion Incidence of early cardiac dysfunction after standard dose anthracyclines in an adult Sub-Saharan population is 27.7% at a mean follow-up of 14.9 months post anthracycline. Routine pre- and post-exposure cardiac assessment should be considered.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Kenji Yodogawa ◽  
Yoshihiko Seino ◽  
Norihiko Ono ◽  
Chikao Ibuki ◽  
Toshihiko Ohara ◽  
...  

Background The outcome of cardiac sarcoidosis (CS) is sometimes fatal. Ventricular tachycardia (VT) is one of the common causes of sudden death in CS patients. In patients with definite diagnosis of CS, corticosteroid therapy should be the absolute indication. However, little is known about the effectiveness for VT. Further, there is no established method for therapeutic evaluation in CS. Thus, we investigated whether or not VT is suppressed following corticosteroid therapy and the utility of Signal Averaged Electrocardiography (SAECG) for therapeutic assessment in CS patients with VT. Methods Fifteen histological proved CS patients presenting with sustained or non-sustained VT were investigated. All of these patients were treated with predonisolone 30mg to 10mg/day, and assessed before and after corticosteroid therapy. All patients underwent SAECG in which the filtered QRS duration (f-QRS), the root mean square voltage of the terminal 40 ms (RMS 40 ) in the filtered QRS complex and the duration of low-amplitude signals < 40μV (LAS 40 ) in the terminal filtered QRS complex were measured. The presence of VT was assessed by Holter monitoring. Results VT was suppressed in 6 patients {VT (−) group} and the remaining 9 patients were not {VT (+) group}. Accumulation of gallium-67 was detected more frequently in VT (−) group than in VT (+) group (66.7% vs. 11.1%, p<0.05). Left ventricular ejection fraction (LVEF) was significantly higher in VT (−) group than that of VT (−) group (54.1+/−20.1 vs 32.8+/−11.7 p<0.05). In VT (−) group, f-QRS and LAS 40 were significantly decreased and RMS 40 was significantly increased compared with those before corticosteroid therapy (f-QRS: 136.3+/−30.6msec vs 116.8+/−25.4msec, p<0.05 LAS 40 : 68.2+/−24.0msec vs 47.8+/−22.9msec, p<0.05 RMS 40 : 7.2+/−3.3 msec vs 13.3+/−7.6msec, p<0.05). However, SAECG parameters did not change significantly in VT (+) group. Conclusions In the early and viable stage of the disease, corticosteroid therapy was effective for VT in CS. Reversible conduction abnormality detected by SAECG might reflect reversible arrhythmogenic substrate for the occurrence of VT. SAECG is useful for therapeutic evaluation of CS patients with VT.


2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Edward Cheung ◽  
Sarah Ahmad ◽  
Matthew Aitken ◽  
Rosanna Chan ◽  
Robert M. Iwanochko ◽  
...  

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.


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