bluuebunny/crossref_metadata_2025_split huggingface dataset:
The diagnosis of acute myocarditis in emergency (DAME) score: improving diagnostics within the emergency department
Authors: Ciccone M M , Capriati G , Gambacorta S , Antonica C , Scicchitano P 
Published: November 2023

Abstract

Purpose
The final diagnosis of myocarditis is challenging. The aim of our study was to provide the D.A.M.E. (Diagnosis of Acute Myocarditis in Emergency) Score for the fast identification of patients suffering from myocarditis at Emergency Department (ED).

Methods
This was a multicenter, retrospective study involving three centers. All medical records from January 2010 to December 2014 reporting a final discharge diagnosis of myocarditis were considered. One hundred-four patients (mean age: 40.2±16.5 years) were enrolled. Clinical, biochemical and instrumental data were gathered. Data were analysed by means of logistic regression model and factorial analysis. A validation cohort from a fourth center was enrolled.

Results
The final determinants of the DAME score were six: fever, chest pain, erythrocyte sedimentation rate (ESR) &gt; 20 mm/h, C-reactive protein (hs-CRP) &gt;3 mg/L, troponin serum levels &gt;3 ng/L, and left ventricle ejection fraction &lt; 50%. All of them received a specified score ranging from 0 to 4. A score &gt; 4 was related to 75% probability of myocarditis; a final score ranging between 1 and 4 was related to 57% probability of myocarditis. ROC curve on the validation cohort (289 patients, 27 with myocarditis) demonstrated the best cut-off to be 7: AUC 0.958 (p&lt; 0.001), sensibility: 100%, specificity: 85.11%, PPV: 40.9%, NPV: 100% (LR+: 6.72; LR-: 0.00). Logistic regression analysis revealed Odds Ratio equal to 2.83 (95% CI 1.90 - 4.20, p &lt; 0.0001).

Conclusions
DAME score can offer a reliable tool in ED setting for the evaluation of patients suffering from suspected myocarditis.
Imaging for prevalence assessment of cardiac transthyretin amyloidosis in patients &gt; 60 years hospitalized in cardiology : the IMPACT study
Authors: Beard E , Alos B , Khalifa B , Rosenberg T , Lequeux B , Ragot S , Saulnier P J , Boucher C , Christiaens L P , Bouleti C 
Published: November 2023

Abstract

Introduction
Transthyretin cardiac amyloidosis (ATTR-CA) is a severe condition with poor prognosis if left untreated (1). Its true prevalence remains unknown because of frequent underdiagnosis, and most published series have focused on highly selected populations (2-3). The objectives of this prospective study were 1) to determine the prevalence of ATTR-CA in consecutive patients &gt; 60 years hospitalized in cardiology wards and presenting with left ventricular hypertrophy (LVH) ≥ 12 mm on transthoracic echocardiography (TTE), using a systematic 99mTc-HMDP bone scintigraphy for diagnosis screening 2) to analyze the characteristics associated with the final diagnosis of ATTR-CA. Population & methods: Between october 2021 and june 2022, 251 patients with echocardiographic LVH (from any cause, including high blood pressure or aortic stenosis) and without any previous diagnosis of CA, were prospectively included and underwent 99mTc-HMDP bone scintigraphy. A monoclonal gammapathy was systematically sought-after to exclude light-chain cardiac amyloidosis (AL-CA) (4). Genetic analysis of the TTR gene was systematically performed in case of definite diagnosis of ATTR-CA. The design of the trial is provided in Figure 1. This is an interim analysis of a private funded study currently being finalized, with a target of 400 patients to be included.

Results
Among the 251 patients, definite ATTR-CA was diagnosed in 17 patients (6.8%), all of them being wild-type. No AL-CA was identified. The 2 subgroups of patients with the highest prevalence of ATTR-CA (&gt;10%) were patients ≥ 65 years with either heart failure or bilateral carpal tunnel syndrom. The majority of diagnosed patients had symptoms or signs suggestive of ATTR-CA, even though CA was never looked-after during their medical pathway, while 30% had no or only one red flag of ATTR-CA. Using multivariable analysis, we found four parameters independently associated with the diagnosis of ATTR-CA as presented in Table 1, one clinical and three echocardiographic, including the alteration of left atrial strain.

Conclusion
We report a high prevalence (6.8%) of ATTRwt-CA in a non-selected cardiological population with LVH from any cause. Most patients were underdiagnosed since they had at least 2 red-flags for ATTR-CA, thus emphasizing the need for continuous and strengthened education on CA. On the other hand, 30% of patients had no obvious sign for CA and the diagnosis was only established by our systematic screening strategy. We report 4 factors significantly associated with ATTR-CA in our population, including the alteration of left atrial reservoir strain. These new data confirm the interest of a complete echocardiographic analysis in case of LVH, to refine the selection of patients who could benefit from 99mTc-HMDP bone scan screening.Study designPredictive factors of ATTR-CA
Immunomodulation to target biopsy-proven myocardial inflammation in genetic cardiomyopathies: a pilot report
Authors: Peretto G , De Luca G , Di Resta C , Campochiaro C , Villatore A , Palmisano A , Vignale D , Busnardo E , Lazzeroni D , Rizzo S , Sala S , Esposito A , Basso C , Dagna L , Della Bella P 
Published: November 2023

Abstract

Background
Preclinical data support the rationale for targeting myocardial inflammation (M-Infl) in genetic nonischemic cardiomyopathies. However, no consistent clinical reports have been provided so far.

Purpose
To provide a pilot report on multimodal detection and targeting of biopsy-proven M-Infl in genetic cardiomyopathies.

Methods
We describe a series of patients (n=25) with genetic cardiomyopathy and M-Infl proven by endomyocardial biopsy (EMB). Multimodal diagnostic workup was extensively applied. Patient-tailored immunomodulatory therapy (IMT) was empirically started to target M-Infl whenever feasible. Multiple outcomes were retrospectively assessed by a dedicated multidisciplinary disease unit.

Results
Patients carrying desmosomal (DGV), cytoskeletal (CGV) and other gene variants, were 12 (48%), 10 (40%), and 3 (12%), respectively. DGV carriers uniformly presented with myocarditis-like chest pain and ventricular arrhythmias, whereas heart failure symptoms were found only in CGV carriers. Dilated cardiomyopathy phenotype and ring-like pattern on cardiac magnetic resonance allowed the best discrimination among genotypes. Noninvasive imaging was concordant with EMB in detecting M-Infl in 21 patients (83%). IMT was started in 17 patients (71%) with no safety issues. By the end of follow-up (median 69 months, range 21-182), signs of M-Infl were documented in 6/18 IMT receivers (33%) and 4/7 untreated cases (57%). For most genotypes, paucity of adverse events was noted while on IMT as compared to the off-treatment period.

Conclusion
Our preliminary data provide a clinical rationale for the research and targeting of M-Infl in a spectrum of genetic nonischemic cardiomyopathies.
Efficacy of cardiac myosin inhibitors for obstructive hypertrophic cardiomyopathy: a systematic review and meta-analysis of randomised controlled trials
Authors: Felix N , Teixeira L M F , Nogueira A K S , Godoi A C , Alexandre F K B 
Published: November 2023

Abstract

Background
Obstructive hypertrophic cardiomyopathy (oHCM) is a hereditary heart disease associated with left ventricular hypertrophy and left ventricular outflow tract (LVOT) obstruction. Cardiac myosin inhibitors (CMI) have emerged as the first disease-specific, non-invasive therapy with promising results in patients with hypertrophic cardiomyopathy. However, its efficacy in the setting of oHCM remains unclear.

Purpose
We aimed to perform a meta-analysis of randomised controlled trials (RCTs) comparing CMI with placebo as a treatment for oHCM in terms of NYHA class improvement, provoked LVOT gradient, and quality of life.

Methods
We systematically searched PubMed, EMBASE, and the Cochrane Library in February 2023 using PRISMA guidelines for RCTs comparing CMI with placebo in oHCM patients. Statistical analyses were performed using R Software version 4.1.0. Between-study heterogeneity was assessed using I2 statistics.

Results
Three RCTs comprising 390 patients were included, of whom 193 (49.48%) were randomised to CMI. CMI were found to be significantly more effective compared with placebo in improving NYHA class (RR 2.23; 95% CI 1.75–2.85; p &lt; 0.001; I2=0%; Fig 1A) and quality of life (SMD 0.64; 95% CI 0.43 to 0.86; p &lt; 0.001; I2=0%; Fig 1B), as well as in reducing provoked LVOT gradients (MD -37.23 mmHg; 95% CI -41.73 to -32.73; p &lt; 0.001; I2=18% Fig 1C).

Conclusion
To our knowledge, this is the first meta-analysis studying CMI versus placebo for the management of oHCM, pointing that CMI were superior to placebo with regards to improvements in NYHA class, quality of life, and LVOT gradients.Figure 1
Myocarditis relapse and COVID19 infection or vaccination: a single-center experience
Authors: Baritussio A , Giordani A S , Basso C , Lorenzoni G , Iliceto S , Marcolongo R , Gregori D , Caforio A L P 
Published: November 2023

Abstract

Background
Myocarditis has rarely been associated with COVID19 infection and it emerged as a possible rare side effect after vaccination with messenger RNA (mRNA) vaccines. Myocarditis may have an unfavorable outcome and may relapse, particularly in females with immune-mediated/autoimmune features and a predisposing immunogenetic background.

Purpose
We aimed to assess the prevalence of myocarditis relapse during CoVid19 outbreak and following the onset of the vaccination campaign in a cohort of patients with history of clinically suspected or biopsy proven myocarditis.

Methods
We included consecutive, randomly selected patients with history of clinically suspected or biopsy proven myocarditis (defined in keeping with the World Health Organization and the European Society of Cardiology (ESC) criteria) coming for a regular follow-up visit to the Cardioimmunology outpatient clinic of a tertiary referral center from September-1-2021 to January-31-2022. History of CoVid19 infection and vaccination status were investigated, and patients underwent clinical, laboratory and echocardiographic check-up (Table1), as per usual practice during planned outpatient follow-up visits. As patients were selected based on already planned follow-up outpatient schedule, to rule out selection bias between the study cohort (n=240) and the remaining patients from our outpatient registry (n=714) we compared clinical, laboratory and imaging features (at baseline and at follow-up) of the two groups.

Results
Out of 240 patients (25% of the 954-outpatient registry cohort) receiving a scheduled follow-up visit, 43 (18%) developed CoVid19 infection, all before vaccination, with mild symptoms; no patient required hospitalization, none developed myocarditis relapse (based upon symptoms, troponin, new ECG or morpho-functional abnormalities at cardiac imaging) during or following CoVid19 infection. Of the 240 patients, 180 (75%) received vaccination, all but 33 with mRNA vaccines; none developed myocarditis relapse, neither early (within 3 weeks from first or second dose) nor later. No differences were found when comparing clinical and diagnostic features between the last follow-up prior to CoVid19 outbreak and the last follow-up by far. There was also no difference in terms of clinical-diagnostic features at baseline and at follow-up between the study cohort (n=240) and the remaining outpatient registry (n=714).

Conclusions
In our randomly selected cohort of patients with history of clinically suspected or biopsy proven myocarditis, both CoVid19 infection and vaccination were uneventful. These data do not support a clinically relevant role of COVID19 infection or mRNA vaccination in triggering myocarditis relapses in patients with prior known myocarditis and reinforce the indication to vaccination also in this patients’ subset, in keeping with a recent ESC consensus document.
Association of epicardial adipose tissue on magnetic resonance imaging with cardiovascular outcomes: Quality over quantity?
Authors: Duca F , Mascherbauer K , Dona C , Koschutnik M , Binder C , Nitsche C , Halavina K , Beitzke D , Loewe C , Bartko P E , Waldmann E , Macherbauer J , Hengstenberg C , Kammerlander A A 
Published: November 2023

Abstract

Background
Quantity of epicardial adipose tissue (EAT) has been associated with poor cardiovascular outcomes. As suggested by computed tomography (CT) studies the quality of EAT may be of incremental prognostic value.
Cardiac magnetic resonance (CMR) is the gold-standard for tissue characterization, however, has never been applied for EAT quality assessment.

Objectives
To investigate EAT quality measured on CMR T1 mapping as predictor of outcome in an all-comer cohort.

Methods
We measured EAT area (EATA) and T1 time (EAT-T1) in a four-chamber views (Figure 1) and tested for association with clinical, demographic, and laboratory parameters using linear regression models. We used Cox-regression analyses to test the association between EATA and EAT-T1 with a composite endpoint of non-fatal myocardial infarction, heart failure hospitalization, and all-cause death.

Results
A total of 966 participants were included (47.2% female, mean age: 58.4years). Mean EATA was 7.3cm2 and mean EAT-T1 was 268ms. On linear regression EAT-T1 was not associated with markers obesity, dyslipidemia or co-morbidities such as diabetes (p&gt;0.05 for all).
During a follow-up of 57.7 months, a total of 280 (29.0%) events occurred. EAT-T1 was independently associated (adj. HR: 1.003, 95%-CI: 1.000 – 1.005, p=0.029, Figure 2) with the composite endpoint when adjusted for established clinical risk factors including age, sex, natriuretic peptide levels, left and right ventricular function, arterial hypertension, body mass index, and coronary artery disease.

Conclusion
In an all-comer CMR cohort, EAT quality, as measured on CMR T1-times, but not EAT quantity is independently associated with a composite endpoint of non-fatal myocardial infarction, heart failure hospitalization, and all-cause death.Figure 1.Regions of interestFigure 2.Kaplan Meier Curves
Sex differences in patients with acute myocarditis: a cohort study
Authors: Buechel J , Balestra G , Haaf P , Marsch S , Kuehne M , Sticherling C , Krisai P 
Published: November 2023

Abstract

Background
Sex differences in patients with acute myocarditis (AM) are poorly understood.

Purpose
We aimed to study sex differences in AM patients including medical history, clinical presentations, diagnostics, and in-hospital outcomes.

Methods
We enrolled 215 patients with a diagnosis of AM at a tertiary hospital between January 2000 and December 2018 into a retrospective cohort study. All patients were hospitalized on an intensive care unit (ICU) with continuous monitoring. We compared sex differences in clinical presentation including ECG, prior medical history, cardiac biomarkers, cardiac imaging (echocardiography and cardiac MRI) and in-hospital outcomes including incident arrhythmia.

Results
Median age was 38 years and 27% were female. Overall, the most common presenting symptoms were chest pain (84%), dyspnea (34%) and fever (29%). Compared to men, women presented more often with dyspnea (47% vs 29%, p=0.015), were older (49 vs 36 years, p&lt;0.001) and had more comorbidities including hypertension (33% vs 18%, p=0.032) and diabetes (14% vs 4%, p= 0.033). In the presenting ECG, female patients were more likely to present with T-wave inversion (46% vs 27%, p=0.017) and less likely with ST elevation (9% vs 39%, p&lt;0.001) compared to male patients. The overall hospital stay was longer in women compared to men (7 vs 5 days, p=0.021) with no difference in the length of ICU stay (2.8 vs 3.0 days, p=0.782). Cardiac biomarkers including high-sensitivity troponin T (median 426 vs 665 ng/l, p=0.415) and CK-MB (median 23 vs 24 U/l, p=0.888) were not different, but women had higher NT-proBNP levels (median 2380 vs 353 ng/l, p=0.032). Left ventricular ejection fraction (LVEF) was similar between sexes, both by transthoracic echocardiography (55% vs 55%, p=0.864) and cardiac MRI (58% vs 56%, p=0.919). During monitoring, atrial fibrillation/atrial flutter occurred in 4% of patients, non-sustained ventricular tachycardia in 15%, sustained ventricular tachycardia in 4% and ventricular fibrillation/cardiac arrest in 2%. While we did not find a sex difference in atrial fibrillation/atrial flutter, women tended to have more severe ventricular arrhythmia (Figure).

Conclusion
Women constituted a quarter of our AM cohort. Compared to men, women were older at the time of AM diagnosis, had more comorbidities, showed different clinical and ECG findings, and were hospitalized longer. While LVEF was similar, women had higher levels of NT-proBNP.Central Illustration
Risk of myocarditis requiring hospitalization following Covid-19 mRNA booster vaccinations
Authors: Arbel R , Yechezkel M , Shmueli E , Yamin D 
Published: November 2023

Abstract

Background
Reports have suggested an association between the development of myocarditis and the receipt of the primary series of messenger RNA (mRNA) Covid-19 vaccines. This association seems to decrease in the first monovalent booster. However, whether this association exists between the second monovalent and first bivalent boosters is unknown.

Purpose
To evaluate the association of the BNT162b2 mRNA booster vaccines and myocarditis events requiring hospitalization.

Methods
We analyzed the medical records of all CHS members aged 12 and above of Clalit Healthcare Services who received the first, second monovalent and bivalent BNT162b2 mRNA COVID-19 vaccine boosters between July 30, 2021, and November 15, 2022. We evaluated the risk differences per 10,000 subjects of myocarditis between 28 days before vaccination (baseline period: 35 days until 8 days before vaccination) and after vaccination (post-vaccination: the day of inoculation until day 27) in a pairwise method using the non-parametric percentile bootstrap. The results were stratified by sex and age.

Results
Among 1,965,200 subjects vaccinated with the first booster, 13 events were recorded post-vaccination versus 2 pre-vaccination (risk difference: 0.46; 95% CI: 0.06-0.10). Among 420,888 subjects vaccinated with the second booster, 4 events were recorded post-vaccination versus 1 pre-vaccination (risk difference: 0.07; 95% CI: -0.02-0.19). Among 131,312 subjects vaccinated with the bivalent booster, 0 events were recorded post-vaccination versus 1 pre-vaccination (risk difference -0.08; 95% CI: -0.23-0.00). Table 1 details the results by sex and age group.

Conclusions
The first BNT162b2 booster vaccine was associated with an increased but rare risk for hospitalization events for myocarditis. This association was not observed in the second and bivalent boosters.
Genetic testing in inflammatory cardiomyopathy/myocarditis: a red flag for cardiomyopathy-like histologic findings
Authors: Bueno Marinas M , De Gaspari M , Baritussio A , Cason M , Celeghin R , Pinci S , Giordani A , Carturan E , Rizzo S , Tarantini G , Marcolongo R , Iliceto S , Basso C , Caforio A L , Pilichou K 
Published: November 2023

Abstract

Background
Myocarditis/inflammatory cardiomyopathy is characterized by the presence of an inflammatory infiltrate in the myocardium. Its etiology is heterogeneous, predominantly mediated by viral infection, as well as a wide variety of toxic substances and drugs and systemic immune-mediated diseases. Outcome could vary from a complete recovery to a residual myocardial injury with the subsequent development of dilated cardiomyopathy (DCM). The factors that play a role in the disease progression are not clear, but it has been shown that inflammatory cardiomyopathy complicated by left ventricular dysfunction, heart failure or arrhythmia is associated with a poor prognosis.

Purpose
To characterize the genetic background of inflammatory cardiomyopathy affected patients to determine the prevalence of pathogenic/likely pathogenic (P/LP) variants in cardiomyopathy-related genes and correlate to histopathologic findings in endomyocardial biopsies (EMB).

Methods
Eighty-nine myocarditis-affected patients (mean age 43±14, 55 males) underwent genetic screening of 200 genes related to inherited cardiomyopathies. Definite/moderate gene association with DCM and Arrhythmogenic Cardiomyopathy was based on the ClinGen framework. Variant prioritization was carried out using American College of Medical Genetics and Genomics rules. Correlation with histopathologic findings of the endomyocardial biopsy and family history was appraised.

Results
Twenty of the 89 suspected myocarditis patients (22%) carried a P/LP variant in cardiac-related genes: 8 in Titin, 3 in Myosin Heavy Chain 7, 2 in Desmoplakin, 2 in Lamin A/C; and Troponin T2, Filamin-C, Myosing Binding Protein C3, Desmin and Sodium Voltage-Gated Channel Alpha Subunit 5 carried one variant each. A re-analysis of the EMBs revealed that 82% of patients showed cardiomyopathy-like features at first histologic findings, with fibrosis in the myocardium and DCM-like phenotype at the second EMB. Further, 13 of the genotype-positive patients referred positive family history for cardiomyopathy and/or sudden cardiac death, whereas only 7 genotype-negative patients declared family history, showing a significant difference among both groups (p-value&lt;0,0001).

Conclusion
The prevalence of P/LP variants in inflammatory cardiomyopathy/myocarditis patients is 22%, most of them associated with DCM. Cardiomyopathy-like features were already observed at the first EMB during the histologic re-evaluation in the majority of genotype-positive patients, and among them, high frequency of positive family history was observed. These findings suggest that genotype-positive inflammatory cardiomyopathy/myocarditis affected patients should be deeply clinically evaluated in order to guarantee an appropriate management for them and their families.