scholarly journals Manifestations and Outcome of Cardiac Sarcoidosis and Idiopathic Giant Cell Myocarditis by 25‐Year Nationwide Cohorts

Author(s):  
Hanna‐Kaisa Nordenswan ◽  
Jukka Lehtonen ◽  
Kaj Ekström ◽  
Anne Räisänen‐Sokolowski ◽  
Mikko I. Mäyränpää ◽  
...  

Background Cardiac sarcoidosis (CS) and giant cell myocarditis (GCM) share many histopathologic and clinical features. Whether they are parts of a one‐disease continuum has been discussed. Methods and Results We compared medical record data of 351 CS and 28 GCM cases diagnosed in Finland since the late 1980s and followed until February 2018 for a composite end point of cardiac death, aborted sudden death, and heart transplantation. Heart failure was the presenting manifestation in 50% versus 15% ( P <0.001), and high‐grade atrioventricular block in 21% versus 43% ( P =0.044), of GCM and CS, respectively. At presentation, left ventricular ejection fraction was ≤50% in 81% of cases of GCM versus in 48% of CS ( P =0.004). The median (interquartile range) of plasma NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide) was 5273 (2782–11309) ng/L on admission in GCM versus 859 (290–1950) ng/L in CS ( P <0.001), and cardiac troponin T exceeded 50 ng/L in 17 of 19 cases of GCM versus in 48 of 239 cases of CS ( P <0.001). The 5‐year estimate of event‐free survival was 77% (95% CI, 72%–82%) in CS versus 27% (95% CI, 10%–45%) in GCM ( P <0.001). By Cox regression analysis, GCM predicted cardiac events with a hazard ratio of 5.16 (95% CI, 2.82–9.45), which, however, decreased to 1.58 (95% CI, 0.71–3.52) after inclusion of markers of myocardial injury and dysfunction in the model. Conclusions GCM differs from CS in presenting with more extensive myocardial injury and having worse long‐term outcome. Yet the key determinant of prognosis appears to be the extent of myocardial injury rather than the histopathologic diagnosis.

2021 ◽  
Vol 5 (3) ◽  
Author(s):  
Ginger Y Jiang ◽  
Qizhe Cai ◽  
E Wilson Grandin ◽  
Marwa A Sabe

Abstract Background Severe cardiac sarcoidosis (CS) can share clinical and histopathologic features with giant cell myocarditis (GCM). Case summary A 56-year-old female presented with 1 week of exertional chest pressure and dyspnoea. Echocardiogram demonstrated extensive regional dysfunction with left ventricular ejection fraction (LVEF) 38%. Cardiac catheterization revealed no obstructive coronary artery disease and cardiac index 1.5 L/min/m2. Cardiac magnetic resonance imaging (MRI) demonstrated diffuse late gadolinium enhancement. Positron emission tomography with fluorodeoxyglucose (FDG) (FDG-PET) computed tomography showed FDG uptake in the anteroseptal and anterior wall and no extracardiac activity. Endomyocardial biopsy (EMB) demonstrated fragments of endocardial fibrosis with mixed inflammatory infiltrate including histiocytic giant cells, which could be due to CS or GCM. She was initially treated for GCM with high dose steroids, tacrolimus, and mycophenolate mofetil. Repeat EMB was pursued and demonstrated multiple granulomas with sharp demarcation from adjacent uninvolved myocardium consistent with CS. A dual-chamber implantable cardioverter-defibrillator was placed, and immunosuppression was changed to prednisone alone with plan for infliximab. Discussion This case illustrates a rare presentation of fulminant isolated CS. Endomyocardial biopsy with sufficient tissue was critical to establish a diagnosis and initiate appropriate immunosuppression.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Bo Hu ◽  
Fei Gao ◽  
Mengwei Lv ◽  
Ban Liu ◽  
Yu Shi ◽  
...  

Abstract Background With the development of cardiac surgery techniques, myocardial injury is gradually reduced, but cannot be completely avoided. Myocardial injury biomarkers (MIBs) can quickly and specifically reflect the degree of myocardial injury. Due to various reasons, there is no consensus on the specific values of MIBs in evaluating postoperative prognosis. This retrospective study was aimed to investigate the impact of MIBs on the mid-term prognosis of patients undergoing off-pump coronary artery bypass grafting (OPCABG). Methods Totally 564 patients undergoing OPCABG with normal courses were included. Cardiac troponin T (cTnT) and creatine kinase myocardial band (CK-MB) were assessed within 48 h before operation and at 6, 12, 24, 48, 72, 96 and 120 h after operation. Patients were grouped by peak values and peak time courses of MIBs. The profile of MIBs and clinical variables as well as their correlations with mid-term prognosis were analyzed by univariable and multivariable Cox regression models. Result Continuous assessment showed that MIBs increased first (12 h after surgery) and then decreased. The peak cTnT and peak CK-MB occurred within 24 h after operation in 76.8% and 67.7% of the patients respectively. No significant correlation was found between CK-MB and mid-term mortality. Delayed cTnT peak (peak cTnT elevated after 24 h after operation) was correlated with lower creatinine clearance rate (69.36 ± 21.67 vs. 82.18 ± 25.17 ml/min/1.73 m2), body mass index (24.35 ± 2.58 vs. 25.27 ± 3.26 kg/m2), less arterial grafts (1.24 ± 0.77 vs. 1.45 ± 0.86), higher EuroSCORE II (2.22 ± 1.12 vs.1.72 ± 0.91) and mid-term mortality (26.5 vs.7.9%). Age (HR: 1.067, CI: 1.006–1.133), left ventricular ejection fraction (HR: 0.950, CI: 0.910–0.993), New York Heart Association score (HR: 1.839, CI: 1.159–2.917), total venous grafting (HR: 2.833, CI: 1.054–7.614) and cTnT peak occurrence within 24 h (HR: 0.362, CI: 0.196–0.668) were independent predictors of mid-term mortality. Conclusion cTnT is a better indicator than CK-MB. The peak value and peak occurrence of cTnT are related to mid-term mortality in patients undergoing OPCABG, and the peak phases have stronger predictive ability. Trial registration: Chinese Clinical Trial Registry, ChiCTR2000033850. Registered 14 June 2020, http://www.chictr.org.cn/edit.aspx?pid=55162&htm=4.


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.


2021 ◽  
Author(s):  
Bo Hu ◽  
Fei Gao ◽  
Mengwei Lv ◽  
Ban Liu ◽  
Yu Shi ◽  
...  

Abstract Background: With the development of cardiac surgery technology, myocardial injury is gradually reduced, but cannot be completely avoided. Myocardial injury biomarkers (MIBs) can quickly and specifically reflect the degree of myocardial injury. Due to various reasons, there is no consensus on the specific values of MIBs in evaluating postoperative prognosis. This retrospective study was aimed to investigate the impact of MIBs on the mid-term prognosis of patients undergoing off-pump coronary artery bypass grafting (OPCABG).Methods: Totally 564 patients undergoing OPCABG with normal courses were included. Cardiac troponin T (cTnT) and creatine kinase myocardial band (CK-MB) were assessed within 48 hours before operation and at 6, 12, 24, 48, 72, 96 and 120 hours after operation. Patients were grouped by peak values and peak time courses of MIBs. The profile of MIBs and clinical variables as well as their correlations with mid-term prognosis were analyzed by univariable and multivariable cox regression models.Result: Continuous assessment showed that MIBs increased first (12 hours after surgery) and then decreased. The peak cTnT and peak CK-MB occurred within 24 hours after operation in 76.8% and 67.7% of the patients respectively. No significant correlation was found between CK-MB and mid-term mortality. Delayed cTnT peak (peak cTnT elevated after 24 hours after operation) was correlated with lower Ccr (69.36±21.67 vs. 82.18±25.17 ml/min/1.73 m2), body mass index (24.35±2.58 vs. 25.27±3.26 kg/m2), less arterial grafts (1.24±0.77 vs. 1.45±0.86), higher Euroscore II (2.22±1.12 vs.1.72±0.91) and mid-term mortality (26.5 vs.7.9 %). Age (HR: 1.067, CI:1.005-1.132), left ventricular ejection fraction (HR: 0.950, CI:0.910-0.992), New York Heart Association score (HR: 1.833, CI:1.155-2.908), total venous grafting (HR: 2.833, CI:1.054-7.611) and cTnT peak occurrence within 24 hours (HR: 0.356, CI:0.731-2.513) were independent predictors of mid-term mortality.Conclusion: cTnT is a better indicator than CK-MB. The peak and peak occurrence of cTnT are related to mid-term mortality in patients undergoing OPCABG, and the peak phases have stronger predictive ability.


2021 ◽  
Vol 8 ◽  
Author(s):  
Shangyu Liu ◽  
Lihui Zheng ◽  
Lishui Shen ◽  
Lingmin Wu ◽  
Yan Yao

Aims: Giant cell myocarditis (GCM) is a rare, rapidly progressing cardiomyopathy with high mortality, if not diagnosed and treated in time. We analyzed the progression and clinical manifestations of patients with definitive diagnosis of GCM.Methods and Result: We enrolled 12 patients diagnosed with GCM in the explanted heart during heart transplantation (HTx) or by endomyocardial biopsy (EMB) and collected information on demographic data, cardiac structure and function, arrhythmias, preliminary diagnosis, and delay of the diagnosis. Seven cases were diagnosed from biopsy samples during HTx, and five cases were diagnosed through EMB. Before the diagnosis of GCM based on pathological analysis, these patients had been incorrectly diagnosed with arrhythmogenic right ventricular cardiomyopathy (n = 5), dilated cardiomyopathy (n = 2), ventricular tachycardia (n = 2), viral myocarditis (n = 1), cardiac amyloidosis (n = 1), and ischemic cardiomyopathy (n = 1) based on clues such as symptoms, arrhythmia, and cardiac imaging. Patients diagnosed with GCM through EMB had a shorter symptom-onset-to-diagnosis time (6.6 ± 2.7 months) and milder heart damage (left ventricular ejection fraction, 47.2 ± 8.8%) than those diagnosed during HTx (11.0 ± 3.3 months, P = 0.034; 31.4 ± 10.9%, P = 0.024).Conclusion: GCM is easily misdiagnosed as other types of myocarditis and cardiomyopathy. Pathological examination of the myocardium is the most reliable diagnostic method for GCM. Endocardial biopsy can identify patients with GCM at an earlier stage.


Cardiology ◽  
2016 ◽  
Vol 135 (1) ◽  
pp. 36-42 ◽  
Author(s):  
Elena Z. Golukhova ◽  
Olga Gromova ◽  
Marina Grigoryan ◽  
Vadim Merzlyakov ◽  
Konstantin Shumkov ◽  
...  

Background: Prediction and potential prevention of sudden cardiac death (SCD) due to malignant ventricular arrhythmia (MVA) represent an obvious unmet medical need. We estimated the prognostic relevance of numerous biomarkers associated with future MVA development in patients with coronary artery disease (CAD) over 2 years of follow-up. Methods: Patients with stable documented CAD (n = 97) with a mean age of 61 ± 10 years were prospectively enrolled in a single-center observational cohort study. Heart failure was diagnosed in 68% of the patients (NYHA class II-III). The mean left ventricular ejection fraction (LVEF) was 50 ± 13%, while 20% of patients had LVEF ≤35%. Sixty-two patients underwent myocardial revascularization during the follow-up (mean 25 ± 11 months). Clinical characteristics (age, gender, diabetes, history of coronary disease and arrhythmias, prior interventions and antecedent medications), noninvasive electrophysiological markers [microvolt T-wave alterations, signal-averaged electrocardiography, QT interval duration and alteration, and heart rate turbulence (HRT) and HR variability], laboratory indices [serum creatinine and creatinine clearance, brain natriuretic peptide (BNP), NT-proBNP, and C-reactive protein and troponin T levels] were assessed with regard to the MVA prognosis. Results: MVA was diagnosed in 11 patients during the prospective follow-up. Prior percutaneous coronary intervention (p < 0.05), MVA or syncope (p < 0.05), on-pump coronary artery bypass grafting during follow-up (p < 0.01), LVEF ≤47% (p < 0.01), a left atrium size ≥4.7 cm (p < 0.05), left atrium index (p = 0.01), filtered QRS duration (p < 0.05), abnormal HRT (χ2 = 6.2, p = 0.01) or turbulence slope (χ2 = 9.5, p < 0.01), BNP ≥158 pg/ml (p < 0.01) and NT-proBNP ≥787 pg/ml (χ2 = 4.4, p < 0.05) were significantly associated with MVA risk by univariate analysis. However, only prior MVA or syncope [odds ratio (OR) 11.1; 95% confidence interval (CI) 2.8-44.4; p < 0.01], abnormal HRT (ОR 13.6; 95% CI 2.8-66.1; p < 0.01) and plasma BNP (ОR 14.3; 95% CI 3.2-65.0; p < 0.01) remained independent predictors of MVA occurrence by multivariate Cox regression analysis. Conclusion: Prior syncope or MVA, HRT and elevated plasma BNP were independent MVA predictors, advocating for the prospective screening of high-risk CAD patients for potential SCD awareness.


2021 ◽  
Vol 59 (1) ◽  
pp. 51-57
Author(s):  
Daniela Maria Cardinale ◽  
Martina Zaninotto ◽  
Carlo Maria Cipolla ◽  
Claudio Passino ◽  
Mario Plebani ◽  
...  

AbstractDrug-induced cardiotoxicity is a major clinical problem; cardiotoxic drugs may induce both cardiac dysfunction and myocardial injury. Several recent studies reported that cardiac troponins measured with high-sensitivity methods (hs-cTn) can enable the early detection of myocardial injury related to chemotherapy or abuse of drugs that are potentially cardiotoxic. Several authors have some concerns about the standard definition of cardiotoxicity, in particular, regarding the early evaluation of chemotherapy cardiotoxicity in cancer patients. Several recent studies using the hs-cTn assay indicate that myocardial injury may precede by some months or years the diagnosis of heart failure (HF) based on the evaluation of left ventricular ejection fraction (LVEF). Accordingly, hs-cTn assay should considered to be a reliable laboratory test for the early detection of asymptomatic or subclinical cardiotoxic damage in patients undergoing cancer chemotherapy. In accordance with the Fourth Universal Definition of Myocardial Infarction and also taking into account the recent experimental and clinical evidences, the definition of drug-cardiotoxicity should be updated considering the early evaluation of myocardial injury by means of hs-cTn assay. It is conceivable that the combined use of hs-cTn assay and cardiac imaging techniques for the evaluation of cardiotoxicity will significantly increase both diagnostic sensitivity and specificity, and also better prevent chemotherapy-related left ventricular (LV) dysfunction and other adverse cardiac events. However, large randomized clinical trials are needed to evaluate the cost/benefit ratio of standardized protocols for the early detection of cardiotoxicity using hs-cTn assay in patients receiving chemotherapy for malignant diseases.


2021 ◽  
Vol 10 (Supplement_1) ◽  
Author(s):  
J Grand ◽  
K Miger ◽  
A Sajadieh ◽  
L Kober ◽  
C Torp-Pedersen ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): The Danish Heart Foundation Background In acute heart failure (AHF), low systolic blood pressure (SBP) has been associated with poor outcome. Less is known of the risk related to normal versus elevated SBP and interaction with left ventricular ejection fraction. Purpose The aim of the present study was to assess the association between baseline SBP and short- and long-term outcome in a large cohort of AHF-patients. Methods A pooled cohort of four randomized controlled trials investigating the vasodilator serelaxin versus placebo in patients admitted with AHF and an SBP from 125 to 180 mmHg. Endpoints were 180-day all-cause mortality and a short-term composite endpoint (worsening heart failure, all-cause mortality or hospital readmission for HF through Day 14). Left ventricular ejection fraction (LVEF) was categorized into HFrEF (&lt;40%) and HFpEF (= &gt;40%). Multivariable Cox regression was used and adjusted for age, sex, baseline body mass index, HFrEF, serum estimated glomerular filtration rate, allocated treatment (placebo/serelaxin), diabetes mellitus, ischemic heart disease, and atrial fibrillation/flutter. Measurements and Main Results A total of 10.533 patients with a mean age of 73 (±12) years and median SBP of 140 (130-150) mmHg were included within mean 8.2 hours from admission. LVEF was assessed in 8493 (81%), and of these, 4294 (51%) had HFrEF. Increasing SBP as a continuous variable was inversely associated with 180-day mortality (HRadjusted: 0.93 [0.88-0.98], p = 0.004 per 10 mmHg increase) and with the composite endpoint (HRadjusted: 0.90 [0.85-0.95], p &lt; 0.0001 per 10 mmHg increase). A significant interaction was observed regarding LVEF, revealing that SBP was not associated with mortality in patients with HFpEF  (HRadjusted: 1.01 [0.94-1.09], p = 0.83 per 10 mmHg increase), but SBP was associated with increased mortality in HFrEF (HRadjusted: 0.80 [0.73-0.88], p &lt; 0.001 per 10 mmHg increase) (Figure). Conclusions Elevated SBP is independently associated with favorable short- and long-term outcome in AHF-patients. The association between SBP and mortality was, however, not present in patients with preserved LVEF. Abstract Figure. Survival plots by SBP and LVEF


Author(s):  
Sahrai Saeed ◽  
Anastasia Vamvakidou ◽  
Spyridon Zidros ◽  
George Papasozomenos ◽  
Vegard Lysne ◽  
...  

Abstract Aims It is not known whether transaortic flow rate (FR) in aortic stenosis (AS) differs between men and women, and whether the commonly used cut-off of 200 mL/s is prognostic in females. We aimed to explore sex differences in the determinants of FR, and determine the best sex-specific cut-offs for prediction of all-cause mortality. Methods and results Between 2010 and 2017, a total of 1564 symptomatic patients (mean age 76 ± 13 years, 51% men) with severe AS were prospectively included. Mean follow-up was 35 ± 22 months. The prevalence of cardiovascular disease was significantly higher in men than women (63% vs. 42%, P &lt; 0.001). Men had higher left ventricular mass and lower left ventricular ejection fraction compared to women (both P &lt; 0.001). Men were more likely to undergo an aortic valve intervention (AVI) (54% vs. 45%, P = 0.001), while the death rates were similar (42.0% in men and 40.6% in women, P = 0.580). A total of 779 (49.8%) patients underwent an AVI in which 145 (18.6%) died. In a multivariate Cox regression analysis, each 10 mL/s decrease in FR was associated with a 7% increase in hazard ratio (HR) for all-cause mortality (HR 1.07; 95% CI 1.03–1.11, P &lt; 0.001). The best cut-off value of FR for prediction of all-cause mortality was 179 mL/s in women and 209 mL/s in men. Conclusion Transaortic FR was lower in women than men. In the group undergoing AVI, lower FR was associated with increased risk of all-cause mortality, and the optimal cut-off for prediction of all-cause mortality was lower in women than men.


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