bluuebunny/crossref_metadata_2025_split huggingface dataset:
Left atrial strain and long-term risk of atrial fibrillation in hypertrophic cardiomyopathy
Authors: Kim J , Lee H J , Kim H K , Lee S C 
Published: November 2023

Abstract

Background
The prevalence of atrial fibrillation (AF) in hypertrophic cardiomyopathy (HCM) patients is higher than in the general population, and it is associated with an increased risk of adverse outcomes, such as stroke, heart failure, and mortality. The left atrium (LA) plays an important role in maintaining normal cardiac function, and alterations in its structure and function can contribute to the development of AF. LA strain, a surrogate of LA remodeling, has been suggested a predictor of future AF.

Purpose
The aim of this study was to explore risk factors of new onset AF highlighting LA strain in patients with HCM.

Methods
Among a prospective cohort of 865 HCM patients collected at two large tertiary centers, 696 with adequate echocardiographic images to measure LA strain without a history of AF were included in the analysis. LA strain was measured on apical four-chamber view at initial evaluation using vendor-independent speckle-tracking software. The primary endpoint was new onset AF during 10 years after initial evaluation.

Results
The median age was 55.0 years and 74.3% were male. A median value of LA reservoir strain (LARS) was 26.2 % [interquartile range 20.4, 33.3]. Among the 696 patients, 66 had new onset AF 10 years, with a 10-year incidence of 15.8%. The unadjusted risk factors for new onset AF were previous stroke, non-apical HCM, family history of HCM, non-sustained ventricular tachycardia, obstructive HCM, extensive late gadolinium enhancement, larger LA diameter, larger LA volume, and lower LARS. LARS remained an independent predictor of new onset AF in multivariable analysis. The best cut-off value of LARS was 17.9% (hazard ratio 4.00, 95% confidence interval 2.44–6.55, p&lt;0.001). Compared with LARS ≥17.9%, LARS &lt;17.9% was significantly associated with a higher incidence of new onset AF, irrespective of presence of clinical risk factors (Figure 1) and LA enlargement (Figure 2).

Conclusions
In patients with HCM, low LA strain is an independent predictor of new onset AF.
Impact of neuromuscular assessment on arrhythmic risk in LMNA cardiomyopathy
Authors: Pepe E M , Villatore A , Di Resta C , Paiotti E , Molinari L , Cariello M , Souryal G , Della Bella P , Previtali S , Sala S , Peretto G 
Published: November 2023

Abstract

Background
The current Risk Score for Life-Threatening Ventricular Tachyarrhythmias (LTVT) in LMNA cardiomyopathy does not consider the frequent association between cardiac and neuromuscular phenotypes as a part of a multisystemic disease. In particular, the prognostic value of tendon retractions (TR) has been recently described.

Objectives
We aimed to assess the prognostic role of TR in a cohort of probands with LMNA cardiomyopathy undergoing multidisciplinary workup at a referral center.

Methods
We included 28 probands with LMNA cardiomyopathy undergoing regular, prospective follow-up at a national referral center for laminopathies. All patients underwent extensive baseline characterization, including complete neuromuscular examination and systematic assessment of TR. Indications to implantable cardioverter defibrillators (ICD) for the primary prevention of sudden cardiac death were guideline-based. Patients were clustered in tertiles according to their baseline LMNA-Risk Score. The primary endpoint was the 5-year occurrence of LTVT, namely arrhythmic cardiac death, VF, sustained VT, or appropriate ICD shocks.

Results
Of the 28 probands with LMNA cardiomyopathy (mean age 33±15 years, 50% males, mean LVEF 55±14 %), neurological assessment identified 14 patients (50%) with TR, including 8 cases with no other signs of neuromuscular involvement. At baseline assessment, the median LMNA-Risk Score was 10% (IQR 6-20%). By 5-year follow-up, 9 patients (32%) met the primary endpoint, including three patients in the lowest tertiles of risk (median LMNA-Risk Score 23, range 6-15%). While other variables showed no remarkable differences between groups, the prevalence of TR was 78% among cases experiencing LTVT and 37% otherwise. The presence of either baseline LMNA-Risk score at the highest tertile (&gt;17%) or TR was 100% predictive of 5-year LTVT (9/9 vs. 7/19, p=0.003). Results did not change after reassessment by the end of follow-up (15±5 years).

Conclusions
Our preliminary data suggest that the inclusion of TR in risk stratification may result in a better patient selection for primary prevention ICD implant in LMNA cardiomyopathy.
Interleukin-6 plasma levels predict mortality and heart failure events in cardiac transthyretin amyloidosis
Authors: Lugitsch J , Hoeller V , Schwegel N , Santner V , Gollmer J , Kolesnik E , Lipp R , Niedrist T , Rainer P , Zirlik A , Ablasser K , Verheyen N , Zach D 
Published: November 2023

Abstract

Background
Low-grade systemic inflammation is a key component in the pathophysiology of heart failure (HF). In particular, the pleiotropic effects of interleukin-6 (IL-6) signaling are of interest as circulating IL-6 levels are associated with mortality in HF patients.

Purpose
The purpose of this study was to investigate whether IL-6 plasma levels predict mortality and HF events in patients with cardiac transthyretin amyloidosis (ATTR-CM).

Methods
This was an analysis of a prospective single-center cohort study comprising 98 ATTR-CM patients. The endpoint was defined as a composite of all-cause mortality and worsening HF events. HF events included HF related hospitalizations and urgent outpatient clinic visits with concomitant intravenous diuretic treatment. Follow-up data were collected using central medical records. Kaplan-Meier estimates and Cox regression analyses were calculated to assess the association between IL-6 and the composite endpoint. Moreover, receiver operator characteristics (ROC) analysis was performed to evaluate the predictive accuracy of IL-6 for the composite endpoint.

Results
Median age was 79 years (76-81), 86 patients (88%) were male, and 46 patients (47%) received the transthyretin (TTR) stabilizer tafamidis at baseline. Median NT-proBNP levels were 2813 pg/ml (1365-4856), mean estimated glomerular filtration rate (eGFR) was 57.9±17.6 ml/min/1.73m², and median urine protein to creatinine ratio (UPCR) was 122.0 mg/g (89.8-204.5). Median IL-6 plasma levels were 4.9 pg/ml (3.0-9.4). During a median observation period of 21.2 months (11.4-32.4), 27 patiens (28%) reached the composite endpoint. IL-6 levels were associated with the composite endpoint (hazard ratio = 1.028, 95% confidence interval = 1.004 to 1.051; p = 0.012). The association was independent of age, TTR stabilizing medication, log-transformed and eGFR-adjusted NT-proBNP levels, as well as UPCR. The area under the ROC curve for IL-6 was 0.70 (95% confidence interval = 0.59 to 0.81; p = 0.003). The optimal cutoff value was 5.3 pg/ml, as determined by Youden’s index, with a sensitivity of 70% and specificity of 66%. Kaplan-Meier estimates demonstrated a mean survival time of 1030 days (95% confidence interval = 911 to 1150) for the study population. A statistically significant difference between the survival rates of ATTR-CM patients with IL-6 levels ≥5.3 pg/ml and those with IL-6 levels below the threshold was indicated by the log-rank test (p = 0.002).

Conclusion
Circulating levels of IL-6 independently predicted adverse outcomes in patients with ATTR-CM. IL-6 plasma levels may be useful for identifying a high-risk ATTR-CM subpopulation.Tablewith baseline characteristicsFigurewith Kaplan-Meier estimates
Predominance of a rare transthyretin mutation (p.Asp38Glu) in patients with hATTR cardiomyopathy: initial data from the Croatian Transthyretin Cardiac Amyloidosis Registry (CroATTR)
Authors: Sipus D , Loncaric F , Jakus N , Fabijanovic D , Samardzic J , Skoric B , Mustapic I , Bakovic Kramaric D , Vinter O , Raljevic D , Ruzic A , Borovecki F , Milicic D , Cikes M , Planinc I 
Published: November 2023

Abstract

Introduction
Transthyretin amyloidosis (ATTR) is classified as hereditary (hATTR) or acquired (wtATTR). hATTR is rare genetic disease with variable penetrance and phenotype, with cardiomyopathy (CM) and/or neuropathy being most relevant clinical presentations. The most common and investigated mutation in Europe is Val30Met, predominant in endemic areas of Portugal and Sweden. On the contrary, p.Asp38Glu is an extremely rare mutation with only several cases mentioned in a Swedish register and isolated cases in Japan, India and Canada. Described cases were associated with early onset CM which required specific treatment, heart- or combined heart and liver transplantation.
The Croatian Transthyretin Cardiac Amyloidosis Registry (CroATTR) is a national, longitudinal, non-interventional, and both retrospective and prospective ATTR registry. It is designed to include patients with clinically proven hATTR-CM or wtATTR-CM according to the current guidelines, as well as family members with a confirmed TTR mutation.

Purpose
This is the first report from the CroATTR, aimed to analyse baseline characteristics of the currently enrolled patients in the first year of the registry.

Methods
All patient data were collected and managed using the REDCap electronic data capture tool. The collected data included demographic, clinical, diagnostic, laboratory and genetic parameters. The CroATTR registry was approved by the Institutional Ethics Committee, and all patients signed informed consent. 18 adult patients are included in this report, divided into the following groups: wtATTR group, hATTR group (genotype +, phenotype +), hATTR group (genotype +, phenotype -).

Results
The patients in the CroATTR Registry were predominantly male (15/18, 83%) with median age of 51 years [45.5-67] (Table 1.). Five (27.78%) patients have wATTR, seven (38.89%) have hATTR with developed CM (phenotype positive) and six (33.33%) have confirmed mutation of the TTR gene but are phenotype negative. 12 out of 13 confirmed mutations were p.Asp38Glu. Patients with hATTR CM were all male, in NYHA class I-III, with a high proportion of atrial fibrillation and amyloid-associated changes in the electrocardiogram as well as higher values of N-terminal pro-B-type Natriuretic Peptide (NT-proBNP).

Conclusion
The CroATTR Registry is formed to provide insights into the prevalence, phenotypic characteristics and natural course of ATTR in Croatia. In the initial Registry analysis, to the best of our knowledge, we reported the largest number of patients with p.Asp38Glu mutation of transthyretin gene up to date. Notably, all patients with p.Asp38Glu originate from the same geographical region in the south-eastern part of Croatia, which we postulate may be a new endemic area for hATTR.Table 1
Value of left ventricular longitudinal strain in predicting overt heart failure in patients with Chagas disease and preserved left ventricular ejection fraction
Authors: Nunes M C P N , Ferreira A M F , Silva J L P S , Carvalho V T C , Santos-Junior O R S J , Cardoso C S C , Oliveira L C O , Santos A C J S , Cruz D S C , Leite S F L , Jesus A P J , Haikal D S A H , Sabino E C S , Ribeiro A L P R 
Published: November 2023

Abstract

Introduction
Speckle-tracking echocardiography (STE) is an established tool to detect early myocardial contractile impairment. In Chagas disease patients, STE may be helpful to identify subtle myocardial damage with the potential of progression to heart failure.

Purpose
This study aimed to assess the value of STE in predicting heart failure in Chagas disease patients with preserved left ventricular ejection fraction (LVEF).

Methods
Patients with Chagas disease living in endemic area who had preserved LVEF (&gt;50%) were selected for the study. Clinical evaluation, B-type natriuretic peptide (NT-ProBNP), electrocardiogram (ECG), and echocardiogram were performed at enrollment in 2015 to 2016 and in the follow-up visit between 2018 and 2019. Global longitudinal strain (GLS) derived from STE was analyzed offline. Peak GLS was calculated as the mean value from 18 segments of the left ventricle (Figure 1). The outcome was development of heart failure defined by elevation of NT-ProBNP levels, categorized according to age-specific cut-points for heart failure at the second evaluation. Patients with abnormal NT-ProBNP at baseline or those who died between the 2 visits were not included.

Results
A total of 692 were enrolled, mean age was 58 ± 12 years and 495 (72%) were women. Mean LVEF at baseline and follow-up visits were 63.4 ± 4.6% and 59.8 ± 11.2% (p&lt;0.001). The median [IQR] time between visit 1 and visit 2 was 5.4 [4.5 - 5.8] years. Among patients alive and attending the second visit, 48 patients (7%) developed overt heart failure with overall incidence rate of 14.1 events per 1000 patient-years (95% CI 10.4-18.6). On multivariable analysis, independent predictors of new-onset heart failure were male sex (OR 2.30, 95% CI 1.02-5.18), age (OR 1.04, 95% CI 1.01-1.07), QRS duration (OR 1.03, 95% CI 1.01-1.04), left atrial (LA) volume indexed (OR 1.05, 95% CI 1.01-1.09), and GLS (OR 0.90, 95% CI 0.81-0.98). LVEF did not remain in the model. The C-statistic of the final model was 0.80 (95% CI 71-88) showing a good discrimination.

Conclusions
In a cohort of patients with Chagas disease from remote areas with preserved ejection fraction, GLS was an important predictor of progression to overt heart failure, after adjustment for sex, age, QRS duration and LA volume. GLS was associated with further increased NT-ProBNP levels, which is useful to guide heart failure management, irrespective of systolic dysfunction.
Ventricular volume ratio as a novel imaging biomarker for disease prediction in 26,516 UK Biobank participants
Authors: Mccracken C , Abdulelah Z , Szabo L , Neubauer S , Petersen S E , Raisi-Estabragh Z 
Published: November 2023

Abstract

Background/Introduction
Right and left ventricular volumes are established imaging biomarkers for disease discrimination and outcome prediction. Limited research in select disease cohorts suggests additional clinical value from consideration of disproportionate (or imbalanced) dilatation of one ventricle over the other. The prognostic utility of this "ventricular volume ratio" (VVR) has not been tested in large, unselected cohorts.

Purpose
To examine associations of right-to-left and left-to-right VVR with cardiovascular phenotypes, prevalent disease, and incident cardiac outcomes in the UK Biobank.

Methods
The analysis includes 26,516 participants with cardiac magnetic resonance (CMR) available. We calculated right-to-left ventricular volume ratio (RV/LV), and the reciprocal left-to-right ventricular ratio (LV/RV), and identified participants with asymmetry above the 95th percentile of healthy cases. Multivariable linear and logistic regression models were used to calculate associations between ventricular volume asymmetry and sample characteristics, other cardiac imaging features, existing disease and incident disease. Incident disease associations were measured using Cox proportional hazards models adjusted by age, sex, systolic blood pressure, body mass index, smoking, Townsend deprivation score, hypertension, diabetes, hyperlipidaemia and other key imaging metrics (LV mass indexed to body surface area, LV ejection fraction and RV ejection fraction). The average follow-up time was 4.6 years (SD 1.3 years).

Results
Ventricular volume asymmetry in either direction was associated with increased age, lower RVEF, poorer LV global function index, poorer global longitudinal strain and greater maximum wall thickness. While RV-dominant volume asymmetry was associated with increased risk of all-cause mortality (HR= 1.56, 95% CI= [1.08, 2.26], p=0.019), LV-dominant volume asymmetry was found to associate with a much wider array of factors in the UK Biobank population. LV dominance was significantly associated with smoking, hypertension, diabetes, hyperlipidaemia, existing respiratory disease and existing heart disease. In fully-adjusted models, LV dominance was associated with increased risk for ischaemic heart disease (1.46 [1.12, 1.91], p=0.005), non-ischaemic cardiomyopathies (3.62, [1.93, 6.79], p &lt; 0.001) and a two-fold increased risk for heart failure (2.30, [1.63, 3.26], p &lt; 0.001).

Conclusion(s)
LV/RV is easily calculated from existing imaging metrics, and could potentially have important added value as a biomarker in predicting heart disease, incremental to well-established risk factors and imaging metrics.
Myocardial work in systemic right ventricle: new insights on right ventricular adaptation to systolic flow
Authors: Sorice D , Fusco F , Scognamiglio G , Abbate M , Merola A , Palma M , Grimaldi N , Barracano R , Borrelli N , Ciriello G D , Roma A S , Colonna D , Correra A , Romeo E , Sarubbi B 
Published: November 2023

Abstract

Background
Myocardial work (MW) is an innovative tool, which combines global longitudinal analysis with non-invasively assessed ventricular pressure to provide a measure of ventricular performance. This technique has recently been used in subpulmonary right ventricle (spRV), but it has not yet been studied in systemic right ventricle (sRV).

Methods and Results
MW indexes were obtained from ventricular pressure–strain loops in 48 patients with sRV (32 transposition of the great arteries following atrial switch repair and 16 congenitally corrected transposition of the great arteries; mean age 38.5±10years; 56% male) and in 20 healthy volunteers, as a control group of spRV.
Echocardiographic parameters and global longitudinal strain(GLS) analysis demonstrated impaired systolic function in the sRV group (TAPSE: 12.3±3mm, S wave:6.9±2cm/s, fractional area change:29[23-35]% , GLS: -13.4±3%, RV septum GLS: -12±4%, RV free-wall GLS: -14.8±4%).
Accordingly, MW indexes were below normal reference values reported for the left ventricle (global work index (GWI): 1056±331 mmHg%; global constructive work (GCW): 1490±269 mmHg%); and global wasted work (GWW) and global work efficiency (GWE) were mildly increased (245.5±162mmHg% and 87±9%, respectively). In addition, sRV free-wall contributed more than the septum to the global performance (GWI septum 879±318, free-wall 1241±412 mmHg%, p&lt;0.0001; GCW septum 1348±287, free-wall 1626±377 mmHg%, p&lt;0.0001). Notably, segmental pressure–strain loops demonstrated higher wasted work values in dyssynchronous segments (figure). As shown in Table1, MW indexes of performance were significantly higher in sRV compared to spRV (spRV GWI:349.3±99mmHg%; p&lt;0.0001; spRV GCW: 451.1±104mmHg%; p&lt;0.0001), likely reflecting sRV adaptation to increased afterload. However, as a response to chronic exposure to systemic pressure, a significant raise of both GWW and GWE in sRV compared to spRV was found (spRV GWW:43.1±22mmHg%, p&lt;0.0001; spRV GWE:92.4±4,p=0.0005).

Conclusion
Assessment of MW in patients with a sRV is feasible and may provide additional data on ventricular global and segmental performance and efficiency, reflecting sRV adaptation to systemic loading.Table 1
Increased cesarean section rate and premature birth according to modified WHO maternal cardiovascular risk in pregnant women with congenital heart disease
Authors: Wedlund F , Von Wowern E , Hlebowicz J 
Published: November 2023

Abstract

Background
During pregnancy and delivery, hemodynamics are altered, and women with adult congenital heart disease (ACHD) might have a higher risk of obstetric and neonatal complications. We sought to investigate pregnancy outcome and complications in relation to complexity of heart condition.

Materials and methods
We studied women with ACHD discussed at multidisciplinary conferences March 2009-May 2021. We studied 149 pregnancies in 101 women. We scored each woman retrospectively according to the modified World Health Organization (mWHO) risk classification and included patients in risk class I (n=36, 24.1%), II (n=43, 28.9%), II-III (n=43, 28.9%), III (n=24, 16.1% and IV (n=3, 2.0%). Relative risks were assessed with logistic regression adjusting for parity and age.

Results
Women with mWHO class ≥III underwent cesarean section more often than women in less complex mWHO classes, p&lt;0.001. Women in mWHO classes I, II and II-III were 83.8% (CI 54.4%-94.2% p&lt;0.001) less likely to give birth prematurely compared to women in mWHO classes III and IV. We found no difference in incidence of preeclampsia, gestational hypertension, gestational diabetes or hemorrhage &gt;1000 ml depending on WHO-class (table 1). Women in mWHO classes III-IV had a higher rate of fetal growth restriction compared to women in mWHO classes I, II, II-III (p&lt;0.007).

Conclusions
Women with complex congenital heart disease (mWHO ≥III) tend to have a higher rate of cesarean section, premature birth and fetal growth restriction.Table 1
Blood and imaging biomarkers in the long-term follow-up of bicuspid aortic valve patients
Authors: Hendriks P , Keuning Z A , Duijnhouwer A L , Meccanici F , Siebelink H J , Van Den Hoven A T , Geenen L W , Eindhoven J A , Baggen V J M , Cuypers J E A A , Kauling R M , Roos-Hesselink J W , Van Den Bosch A E 
Published: November 2023

Abstract

Background
Bicuspid aortic valve (BAV) is a common congenital heart defect. Patients with BAV are at risk for long-term complications such as arrhythmias and reinterventions.

Purpose
This study aimed to investigate (1) sex differences in blood and imaging biomarkers and (2) the role of blood and echocardiographic biomarkers in the long-term risk prediction of patients with BAV.

Methods
In this multicenter prospective observational study, all included adult BAV patients underwent venous blood sampling and transthoracic echocardiography including speckle tracking at the time of inclusion. Analyzed blood biomarkers were: RDW, creatinine, CRP, TroponinT, NT-proBNP and TGF-beta. Sex differences were analyzed at baseline. Associations between blood & echocardiographic biomarkers and arrhythmia-free & intervention-free survival were determined by Cox proportional hazards models.

Results
A total of 182 patients were included (median age 34 (Interquartile range: 23-46) years, 55.5% male). Significant differences were found in blood biomarkers at baseline between men and women. CRP, NT-proBNP and RDW were higher in women while creatinine, TroponinT and TGF-beta were higher among men (figure 1). After a median follow-up time up of 6.9 (IQR: 6.5- 9.9) years, arrhythmia-free and event-free survival rates were respectively 81.0% and 73.1%. NT-proBNP was the only blood biomarker independently associated with both arrhythmia free and intervention free survival. On echocardiography an increase in LA size, LVEDD, LV mass index and E/E’ratio were associated with arrhythmia free survival. Intervention free survival was associated with increased LA size, LV mass index, aortic valve peak velocity and the presence of moderate or severe aortic regurgitation. No associations were found with ejection fraction and global longitudinal strain (figure 2).

Conclusion
Significant sex-related differences were observed in blood biomarkers between men and women with BAV at baseline. NT-proBNP was the only blood biomarker associated with arrhythmia-and intervention-free survival. The strongest associations of echocardiographic markers were those indicating diastolic function and LV mass index.Sex differences in blood biomarkersForest plot