bluuebunny/crossref_metadata_2025_split huggingface dataset:
Acute myocarditis presentation and intra-hospital prognosis: results of the multicenter prospective MYOCARDITIRM study
Authors: Bouleti C , Bejan-Angoulvant T , Lairez O , Genet T , Tea V , Alos B , Bonnet G , Jacquier A , Latucca B , Mousseaux E , Huttin O , Lim P , Redheuil A , Angoulvant D , Ou P 
Published: October 2024

Abstract

Background
Acute myocarditis (AM) usually presents as chest pain with rising troponin and normal coronary arteries. Despite frequent favorable evolution at the acute phase, it is associated with heart failure and ventricular rhythm disorders and is considered the leading cause of sudden cardiac death in young apparently healthy adults. There is no recommendation for AM, only expert consensus, given the lack of important databases for a strong level of confidence.

Methods
We performed a multicenter prospective observational study of patients admitted for AM acknowledged by cardiac MRI to describe baseline characteristics, management, in-hospital and long-term outcomes of AM patients in France. Secondary objectives were to investigate determinants of prognosis, modalities of treatment and follow-up, and to identify gaps between expert consensus and real-life management.

Results
We enrolled 805 consecutive patients hospitalized for AM in 49 participating centers between May 2016 and February 2019. Median age was 31 (IQR 23-41) with 19% of female. Chest pain was the most frequent symptom (93%) and 112 (14%) had severity criteria upon admission (LVEF&lt;50% or severe ventricular arrythmia or high-grade AV block or cardiogenic shock). ST elevation was observed in 49% of patients. One 26-years old patient died during initial hospital stay. Overall, 62 patients (8%) experienced intra-hospital complication of their AM, defined as a combined endpoint of death, LVEF&lt;40% (MRI), sustained ventricular or supraventricular arrythmia, acute renal failure, cardiogenic shock, need for ventricular assistance device, need for inotropic drugs, temporary cardiac pacing, pace-maker or cardiac defibrillator implantation. Patients with initial severity criteria were 10 times more likely to present intra-hospital complication (33% vs 3.6%, p&lt;0.001).

Conclusion
This prospective cohort represents the largest database on AM worldwide. Here we report baseline characteristics and in hospital events. Most patients had typical presentation with favorable outcomes but as much as 8% of patients did exhibit intra-hospital complication. The prospective follow up will provide valuable data to better identify subset of patients with poor prognosis that may be of interest to develop new therapeutic strategies.Acute myocarditis on cardiac MRI
Adherence to sports counselling in middle-aged individuals with myocarditis
Authors: Schuetze J , Bernhard B , Stark A W , Boscolo M , Pavlicek M , Graeni C 
Published: October 2024

Abstract

Introduction
Although current guidelines recommend abstaining from sports for a duration of a minimum of three months in patients with myocarditis, the implementation of this recommendation and its adherence to it are unknown.

Objective
Investigate counselling and adherence to sports behaviour in patients with myocarditis.

Methods
A cohort of patients diagnosed with myocarditis, based on the ESC criteria, received questionnaires three months post-diagnosis to assess their activity levels before and after the diagnosis as well as the adherence to sports counselling.

Results
In our study, 46 myocarditis patients (11; 25% women) with a mean age of 52±14.5years were analysed. At the time of diagnosis, 39 patients (84%) reported physical activity, with 7 (15%) engaging in competitive sports. Following myocarditis diagnosis, 27 (60%) were recommended to abstain from sports or reduce physical activity, with 92% adherence. Of those 11 (23%) were physically active without receiving recommendations continued physical activity (p&lt; 0.001). After three months, less patients were physically active (i.e. 25; 54% patients) compared to 39 (84%) patients at baseline (p = 0.038), 13 of those being physically active but with reduced exercise intensity according to recommendations. Of those who continued exercise without changes (n=13, 28%), only 2 (4%) reported having received recommendations. All competitive athletes (7, 15%) received counselling, whereas significantly fewer recreational athletes 19 (41%) did (p = 0.039). Women received numerical non-significant fewer counselling (4, 36% of all women, compared to 23, 67% of all men; p = 0.186).

Conclusion
Middle-aged individuals with suspected myocarditis exhibit excellent adherence to recommendations regarding physical activity. However, a notable proportion did not recall receiving counselling on exercise and physical activity. This highlights the need for clinicians to provide comprehensive counselling to all patients with suspected myocarditis consistently.Illustrating the sports behaviourcomparing activity at 0 and 3 months
The relationship between serum lumican levels and myocardial fibrosis in patients with hypertrophic cardiomyopathy
Authors: Hancioglu E , Ozcan S , Donmez E , Okuyan E , Sahin İ 
Published: October 2024

Abstract

Background
Hypertrophic cardiomyopathy (HCM) is the leading cause of sudden cardiac death in young individuals and an important cause of heart failure at any given age. Lumican, is an indicator of fibrosis and has been studied in various cardiac conditions. In this study, we aimed to evaluate the relation between serum Lumican levels and presence and extent of myocardial fibrosis in HCM.

Methods
The patients diagnosed with HCM between June 2022 and March 2023 were enrolled consecutively in this prospective study. Age and gender-matched healthy individuals formed control group. Additionally, HCM patients divided into two groups according to extent of late gadolinium enhancement (LGE).

Results
A total of 114 patients (62.3% male, 54.5±9.4 mean age) were enrolled in this prospective study. 64 patients formed HCM (+) and 50 patients formed HCM (-) group. The HCM (+) group is divided into two groups as LGE (+) and LGE (-) according to LGE involvement. Serum Lumican [p=0.018, β: 1.561, OR (95% CI): 1.080-2.257], NT-proBNP [p=0.043, β: 1.209, OR (95% CI): 1.079-2.527] and maximum wall thickness (MWT) [p=0.033, β: 1.322, OR (95% CI): 1.023-1.707] were revealed as independent risk factors associated with LGE by multivariate logistic regression analysis (Figure 1). ROC curve analysis was performed to identify the optimal cut-off value and calculate area under the curve (AUC) for Lumican, troponin (Tn) and NT-proBNP in order to predict the presence and extent of LGE in patients with HCM. A cut off value of 9.06 for Lumican was associated with 67.8% sensitivity and 65.5% specificity in prediction of LGE. A cut-off value of 932.4 for NT-proBNP was associated with 60% sensitivity, 60% specificity in prediction of LGE.

Conclusion
Myocardial fibrosis is one of the important mechanisms in HCM pathophysiology. Although LGE on cardiac magnetic resonance imaging(CMR) allows comprehensive evaluation of myocardial fibrosis, the support of diagnosis with a biomarker would be beneficial in clinical practice.Our study revealed that serum Lumican level is an independent predictor of severe LGE in HCM patients. Lumican can be used in addition to CMR for evaluating extence of myocardial fibrosis in HCM patients and may help clinicians in guiding follow-up and treatment.
Anderson-Fabry disease patients with anatomical heart hypertrophy express a higher degree of electrocardiographic hypertrophy compared to hypertrophic cardiomyopathy patients
Authors: Di Nicola F , Barile L , Schiavo M A , Sena G , Ruotolo I , Catalano C , Parisi V , Fanciullo N , Barlocco F , Lillo R , Re F , Graziani F , Galie' N , Olivotto I , Biagini E 
Published: October 2024

Abstract

Background
Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disease that has progressively gained attention due to the availability of specific therapeutic options. Heart involvement is of critical prognostic significance and differential diagnosis with Hypertrophic Cardiomyopathy (HCM) is as challenging as it is crucial to provide the patient with the appropiate treatment.

Purpose
To describe ECG features in AFD patients according to the presence of anatomical hypertrophy and compare them with matched HCM patients.

Methods
One-hundred-sixty-two AFD patients were recruited in the present multicentre study cohort and divided into 2 groups according to the presence (Group A, n=111) or the absence (Group B, n=51) of anatomical hypertrophy. Group A patients were then compared with a matched cohort of HCM patients (n=111) with the use of Propensity Score Martching relatively to sex, age and maximal wall thickness.

Results
AFD patients with cardiac hypertrophy, compared with matched HCM patients with the same indexed cardiac mass at echocardiography [151 (124-211) vs 145 (123-182) g/m2; p=0.1675], showed on the surface electrocardiogram a higher degree of electrical hypertrophy expressed as increased total QRS score [204 (158-271) vs 177 (138-221) mm; p=0.0004] and R wave amplitude in aVL ≥ 11 mm [51 (45.9%) vs 25 (22.5%); p&lt;0.0001], whereas they did not differ with respect to Sokolow-Lyon index [31 (18-44) vs 32 (22-40) mm; p=0.518] and Cornell index [19 (12-25) vs 17 (12-24) mm; p=0.5].
Furthermore, AFD patients featured wider QRS complexes [110 (100-134) vs 100 (90-106) ms; p&lt;0.0001] and a higher prevalence of Right Bundle Branch Block (RBBB), both complete [25 (22.5%) vs 3 (2.7%); p&lt;0.0001] and incomplete [17 (15.4%) vs 2 (1.8); p&lt;0.0001], as well as non specific intraventricular block [14 (12.6%) vs 4 (3.6%); p&lt;0.0001].

Conclusions
Our cohort of Anderson-Fabry patients, compared with HCM patients with the same indexed cardiac mass at echocardiography matched by age, sex and wall thickness, featured a higher degree of electrocardiographic hypertrophy expressed as increased total QRS score and R wave amplitude in aVL, whereas it did not differ with respect to Sokolow-Lyon and Cornell indexes. These findings may prove beneficial in achieving earlier the diagnosis of AFD, enabling clinicians to initiate swiftly and without delay the specific disease modifying therapies available.Electrocardiographic differencesTotal QRS Score AFD vs HCM
Sex differences in paediatric onset hypertrophic cardiomyopathy
Authors: Norrish G , Hall K , Field E , Boleti O , Cervi E , Kaski J K 
Published: October 2024

Abstract

Background
Hypertrophic Cardiomyopathy (HCM) is a heterogeneous disease characterised by age related incomplete penetrance and variable long term outcomes. Sex differences have been described in both the clinical phenotype and natural history of adults with the disease, which have been attributed to both biological (eg sex hormone effect) and non-biological (eg societal and cultural effects) factors. It is unknown if similar sex differences exist in childhood-onset HCM.

Purpose
This study aimed to investigate the influence of biological sex on the clinical characteristics and outcomes of children with HCM.

Methods
Retrospective cohort study from an international registry of patients diagnosed under the age of 18 years with HCM. Patients with syndromic disease (RASopathy syndromes, inborn errors of metabolism, neuromuscular disease) were excluded. Sex differences in baseline characteristics and clinical outcomes were described. Primary outcomes were all-cause mortality or cardiac transplantation, Major Arrhythmic Cardiac Event (MACE) and heart failure event (defined as heart failure death or cardiac transplantation)

Results
Of 1262 patients, 851 (67.4%) were male (median age 11 yrs (IQR 7.4, 14.4) vs 11.1 (IQR 6.9, 14.0) in females, p=0.0312). Female patients were more likely to have a sarcomeric variant identified (n=208, 85.6% vs 388 (79.4%), p=0.041) despite equivalent genetic testing utilisation between sexes. Female patients were more likely to have severe heart failure symptoms (NYHA 3 or 4) (n=19, 6.1% vs n=20, 2.5% p value 0.030) but there was no difference in the proportion on medical therapy (male n=328, 39.7%, female n=167, 41.8, p value 0.476). The clinical phenotype in terms of degree of hypertrophy, left atrial size and left ventricular outflow tract gradient did not differ by sex. Over a median follow up of 5.2 years (IQR 2.4, 9.9) all-cause mortality or cardiac transplantation did not vary by sex but females were more likely to experience a heart failure event (females 0.6/100,000 pt years (95% CI 0.39-0.92) vs males 0.32/100,000 pt years (95% CI 0.21 – 0.50), hazard ratio 1.89, p value 0.0467). The incidence of MACE did not differ by sex but there was a trend towards higher implantable cardioverter defibrillator implantation in females (n=123, 30.7% vs n=210, 25.4%, p value 0.051).

Conclusions
For the first time, this study has shown sex differences in paediatric-onset hypertrophic cardiomyopathy. Despite no difference in baseline clinical phenotype, young female patients are more likely to experience heart failure symptoms and heart failure events over follow up. Further studies are required to explore the underlying mechanisms for these observed differences.Table summarising outcomes by sexSurvival free from heart failure
Improved quantification of coronary stenosis in patients with high calcium scores using spectral reconstructions in coronary CT angiography
Authors: Monteagudo Ruiz J M , Martinez Vives P , Garcia Sebastian C , Carrion Sanchez I , Garcia Linacero L M , Arteagoitia Bolumburu A , Garcia Martin A , Hinojar Baydes R , Casas Rojo E , Jimenez Nacher J J , Fernandez Mendez M A , Arribas Marcos A , Zamorano Gomez J L , Fernandez-Golfin Loban C 
Published: October 2024

Abstract

Background
Coronary CT angiography has demonstrated reduced accuracy in patients with high calcium scores due to blooming artifacts, often resulting in overestimated stenosis.

Purpose
This study aimed to assess the impact of spectral reconstructions on coronary stenosis quantification in patients with high calcium scores (&gt;500), utilizing invasive coronary angiography as the gold standard.

Methods
Twenty consecutive patients referred for coronary artery disease assessment underwent both coronary CT angiography and invasivecoronary angiography. Conventional 120 kV images, 100 keV virtual monoenergetic images, and iodine-no-water images were reconstructed. Two blinded operators independently reviewed the images. Lesions were qualitatively classified in both CCTA and invasive angiography as stenosis of 1-24%, 25-49%, 50-70%, and &gt;70%.

Results
A total of 87 plaques were evaluated. Mean age was 72 years (SD=7.7) and 79% were male. Pre-test probability were low, medium and high in 15.8, 26.3% and 57.9% of patients respectively. Mean calcium score was 1662, with an average dose of 7.5 mSv (67.9% retrospective studies). Nitrates were administered in 89.5% of patients, and 47.4% received intravenous beta-blockers. Sensitivity, specificity, positive predictive value, and negative predictive value to detect lesions &gt; 50% improved from 94%, 57%, 38%, 97% with conventional images to 94%, 82%, 59%, 98% with 100 keV virtual monoenergetic images.

Conclusion
Spectral reconstructions significantly enhance diagnostic accuracy in coronary CT angiography of severely calcified coronary arteries.
Evaluation of clinical need, effectiveness, and safety of the first myosin inhibitor mavacamten in real world patients
Authors: Seuthe K , Feidakis A , Nies R , Bruewer M , Pennig L , Mons U , Baldus S , Pfister R 
Published: October 2024

Abstract

Background
Hypertrophic cardiomyopathy has a high burden of heart failure if obstruction in the left-ventricular outflow tract (HOCM) is present. Mavacamten is the first available myosin inhibitor specifically addressing the underlying pathophysiology of hypercontractility in HOCM. Based on positive results of respective approval studies, mavacamten is recommended for therapy of HOCM in recent ESC guidelines but clinical real world data are lacking.

Methods
In this ongoing study, consecutive patients with current or previous diagnosis of HOCM presenting at a single referral centre for hypertrophic cardiomyopathy from March 2023 to February 2024 were included and analysed by treatment modality. In patients initiating mavacamten therapy, clinical data of 4 weekly follow ups were assessed, including efficacy (NYHA class, LVOT gradient and biomarkers) and adverse events.

Results
A total of 72 patients were included (59.1 ± 13.0 years, 68% male). Mavacamten treatment was started in 30/72 (41.7%) patients, 3/72 (4.1%) were referred for transcatheter septal reduction therapy (SRT), and 39/72 (54.2%) did not require any escalation in their treatment due to low gradient with current treatment or few symptoms. Of the latter, 3/39 (7.7%) had previous SRT, 36/39 (92.3%) were stable on negative inotropic medication.
The average age of patients selected for mavacamten therapy was 58.0 ± 13.0 years, 80% were male, and the average thickness of the interventricular septum was 18.8 ± 2.8 mm. 22/30 (73.3%) were on baseline therapy with beta-blockers, 3/30 (10.0%) with calcium channel blockers, 2/30 (6.7%) with both, and 4/30 (13.3%) had additional treatment with disopyramide.
As shown in table 1, there was a significant decrease in the LVOT gradient under Valsalva maneuver observed 4 weeks after initiation of mavacamten treatment (p=0.006), while the reduction in peak exercise LVOT gradient was statistically significant after 8 weeks (p=0.02). Simultaneously, a significant decrease in Nt-pro-BNP levels was observed after 4 weeks of therapy (p&lt;0.001). A significant drop in troponin T levels compared to baseline was only noted after 20 weeks of treatment (p=0.02). At baseline, 66.7% patients were classified as NYHA class III/IV, and 12.5% after 20 weeks of treatment. 75% of the patients improved ≥1 NYHA class after 20 weeks.
Regarding adverse events, there was one case of ejection fraction dropping to &lt;50%, which resolved after halting treatment. Additionally, there were two reports of dizziness and one reported syncope, all of which resolved without taking any actions.

Conclusion
In this real-world single centre HOCM cohort, 41.7% of patients had an indication for myosin inhibitor therapy. The safety and effectiveness in lowering the LVOT gradient and improving NYHA class were consistent with findings from the approval studies.
Functional capacity by cardiopulmonary exercise testing is not related to health status in obstructive hypertrophic cardiomyopathy
Authors: Bjerregaard L , Maron M S , Maron B J , Myrte E , Jensen M S K , Rowin E J 
Published: October 2024

Abstract

Background
In obstructive hypertrophic cardiomyopathy (HCM), the primary goal of therapy is to improve patient quality of life by decreasing symptom burden. Increasingly, cardiopulmonary exercise testing (CPET), with peak oxygen consumption (pVO2) and ventilator efficacy (VE/VCO2), is used to assess efficacy of novel therapies in clinical studies and selectively in clinical practice to determine treatments. However, the relationship of CPET variables to symptom burden in HCM is not well established. Therefore, we examined the relationship between CPET variables and health status measured by Kansas City Cardiomyopathy Questionnaire (KCCQ) and NYHA functional classification in a cohort of obstructive HCM patients.

Methods
Consecutive patients with HCM and resting left ventricular outflow tract (LVOT) obstruction (≥50 mmHg) underwent CPET between October 2022 to January 2024. Patients with a peak RER &lt;1.0 were excluded (n=4). To investigate distribution of patient reported health status (KCCQ) and NYHA functional classification, patients were stratified into groups for pVO2 (&lt;14, 14-20, and &gt;20 mL/kg/min) to reflect moderate to severe, mild to moderate, and little to no functional limitation, respectively.

Results
Of 58 patients, clinical evaluation was at 59 ± 13 years of age, 55% male, with LVOT gradient of 83 ± 28 mmHg at rest with 84% NYHA class II or III and 76% with KCCQ overall summary (OS) &lt;75. Both pVO2 and VE/VCO2 had significant but weak correlation with NYHA class and KCCQ scores (Table 1), with the strongest correlation observed with pVO2 and KCCQ-OS (R2= 0.13, p=0.004). In contrast, % predicted pVO2 was not significantly correlated with NYHA or KCCQ. Notably, despite 14 patients being classified as having little to no limitations by pVO2, 29% of these patients had mild to moderate disability and 29% had moderate to severe disability by KCCQ-OS (Figure 1).

Conclusion
In patients with symptomatic obstructive HCM, objective measures of exercise performance, including pVO2 and VE/VCO2, correlate poorly with measurements of health status. These data suggest that CPET results should not be considered a surrogate marker of heart failure symptom burden in obstructive HCM.
Epidemiology and prognosis of obstructive and non-obstructive hypertrophic cardiomyopathy in Finland: a nationwide cohort study
Authors: Laine M , Holsa O , Simin J , Laatikainen O , Vilen S , Kuusisto J 
Published: October 2024

Abstract

Background
Hypertrophic cardiomyopathy (HCM) is an often hereditary myocardial disease. The Finnish population has a specific genetic background, which may influence and even predispose people to various hereditary diseases. Whilst advances in genetic testing and imaging techniques may have influenced the detection of HCM and associated subtypes, contemporary data on incidence and prevalence remain limited.

Purpose
This study aimed to evaluate the current trends in the incidence, prevalence, and prognosis of obstructive and non-obstructive HCM in a nationwide cohort in Finland.

Methods
A population-based registry study was conducted using data from Finnish nationwide healthcare registries (HILMO/avoHILMO) including individuals aged at least 12 years with recorded HCM diagnosis between 2000 and 2021. The incidence was calculated by dividing the number of newly diagnosed HCM patients by the Finnish background population. Furthermore, the mean incidence per age-group was calculated by dividing the sum of age-group specific incidences by the number of age groups. The date and cause of death were obtained from national registries (Statistics Finland). Mortality was computed by dividing the number of deaths by follow-up length. Patients were followed from 2014 to 2021, and incident cases were defined as new diagnoses after 2014. HCM cases were stratified into obstructive HCM and non-obstructive HCM based on ICD-10 codes I42.1 and I42.2, respectively.

Results
Among 5,834 documented patients with HCM, the overall mean incidence by age-group was 2.64/100,000, with incidence increasing with age, peaking in those aged ≥85 years (6.22/100,000 patient-years [PY]). Annual incidence of obstructive HCM decreased from 2.2 to 1.4/100,000 PY, while non-obstructive HCM increased from 4.6 to 5.4/100,000 PY between 2014 and 2021 (Fig 1). Prevalence of non-obstructive HCM rose from 40 to 56/100,000 PY, while obstructive HCM prevalence remained stable (23-24/100,000 PY). All-cause mortality was 5.5/100 PY, with obstructive and non-obstructive HCM mortality at 5.7 and 4.6/100 PY, respectively. HCM-specific deaths accounted for 16% and 12% of all deaths in patients with non-obstructive and obstructive HCM, respectively.

Conclusions
This nationwide study in Finland revealed an increasing incidence and prevalence of non-obstructive HCM over time, contrasting with stable rates among patients with obstructive HCM. Incidence and prevalence were strongly age dependent. Non-HCM-related deaths were predominant in both HCM subtypes.