bluuebunny/crossref_metadata_2025_split huggingface dataset:
Valve-in-valve procedure for bioprosthesis dysfunction in rheumatic patients: a new procedure for an old disease
Authors: Lopes M P , Emer Egypto Rosa V , Palma J H , Fernandes J R C , De Santis A S A L , Spina G S , Abizaid A C , De Brito Jr F S , Tarasoutchi F , Sampaio R O , Ribeiro H B 
Published: October 2021

Abstract

Background
Bioprosthetic heart valve has a limited durability and lower long-term performance especially in rheumatic patients. The standard treatment for degenerated bioprosthesis involves redo open-heart surgery, which is associated with significant morbidity and mortality, particularly in high-risk patients. Minimally invasive procedures, as valve-in-valve (ViV) implantation, may offer an attractive therapy alternative.

Methods
The aim of this study was to evaluate the characteristics and short-term post-procedure data of rheumatic and non-rheumatic patients undergoing aortic, mitral or tricuspid ViV procedure.

Results
Single centre data-base analysis study, with prospective data collection, during the period of May 2015 to September 2020. Among 106 patients included, 69 had rheumatic etiology and 37 were non-rheumatic. Rheumatic patients had higher incidence of female sex (73.9% vs 43.2%, respectively; p=0.004) and atrial fibrillation (82.6% vs 45.9%, respectively; p&lt;0.001). There were no statistical difference regarding number of previous surgeries (2 [3–1] vs 1 [2–1], respectively; p=0.103). There was a tendency towards higher 30-day mortality in rheumatic patients (21.7% vs 5.4%, respectively; p=0.057) and device success rate was low, but similar between groups (78.3% vs 72.0%, respectively; p=0.710). Transapical access rate was also higher in rheumatic group (92.6% vs 70.3%, respectively; p=0.008);

Conclusion
Rheumatic patients tend to have a higher mortality when undergoing ViV procedures, although not statistically significant. Rheumatic patients underwent a greater number of previous valve surgeries than reported in literature, and also had high rate of transapical ViV procedure, which may have contributed to higher 30-day mortality and a lower device success rate.

Funding Acknowledgement
Type of funding sources: None.
Cardiac damage staging classification and prognosis in low flow low gradient severe aortic stenosis
Authors: Snir A , Ng M K , Strange G , Playford D , Stewart S , Celermajer D S 
Published: October 2021

Abstract

Introduction
There are currently no established prognostic models for low-flow, low-Gradient (LFLG) severe aortic stenosis (AS). The Cardiac Damage Staging Classification, has been validated as a clinically useful prognostic tool in high-gradient severe AS, but not specifically in LFLG severe AS.

Objective
To assess and confirm the prognostic utility of the Cardiac Damage Staging Classification, in LFLG severe AS patients.

Methods
We analysed data from the National Echocardiography Database of Australia (NEDA), a national multicentre registry with individual data linkage to mortality. Of 192,060 adults (aged 62.8±17.8 years) with comprehensive ultrasound profiling of the native aortic valve studied between 2000–2019, 12,013 (6.3%) had severe AS. Based on standard echocardiographic parameters, 611 classical and 959 paradoxical LFLG patients were identified. Mean follow-up was 70±41 months. All-cause and cardiovascular-related mortality were assessed for each LFLG group on an adjusted basis (age and sex), according to cardiac damage classification staging.

Results
Paradoxical LFLG patients were younger (mean age 74.3 vs 76.2, p=0.006) and more often female (62.8% vs 36.3%, p&lt;0.001). Classical LFLG patients had greater associated cardiac damage at diagnosis (76% vs. 49%, for Stage ≥2, p&lt;0.001). Compared to Stage 0 paradoxical LFLG patients, adjusted 1- and 5-year cardiovascular-related mortality was 200% (HR 2.82, 95% CI 1.12–7.11) and 120% (HR 2.42, 95% CI 1.46–4.02) higher in Stage 2 patients and 350% (HR 4.23, 95% CI 1.68–10.63) and 175% (HR 3.18, 95% CI 1.90–5.34) higher in Stage 3/4 patients, respectively. Compared to Stage 1 classical LFLG patients, adjusted 1- and 5-year cardiovascular-related mortality was 58% (HR 1.66, 95% CI 0.95–2.90) and 24% (HR 1.35, 95% CI 0.91–1.99) higher in Stage 2 patients and 125% (HR 2.48, 95% CI 1.43–4.28) and 52% (HR 1.87, 95% CI 1.26–2.78) higher in Stage 3/4 patients, respectively.

Conclusion
In patients with LFLG severe AS identified by echocardiography, increasing severity denoted by the cardiac damage staging classification is strongly associated with increasing medium- to long-term mortality.

Funding Acknowledgement
Type of funding sources: None. Stages + Classical LFLG 5 year mortalityParadoxical LFLG 5 year mortality curves
The MIDA quantitative mortality risk score: prognostic model in floppy mitral valves
Authors: Essayagh B , Benfari G , Antoine C , Grigioni F , Le Tourneau T , Roussel J C , Bax J , Delgado V , Ajmone N , Van Wijngaarden A , Tribouilloy C , Hochstadt A , Topilsky Y , Michelena H , Enriquez-Sarano M 
Published: October 2021

Abstract

Background
The Mitral Regurgitation International Database (MIDA) score is a validated tool for Degenerative Mitral Regurgitation (DMR) management, being able to position a given patient within a continuous spectrum of short and long term mortality. However, whether this score may be applicable and incremental in the entire span of Floppy Mitral Valves (FMV), regardless of DMR severity, remains unsubstantiated.

Methods
The MIDA-Quantitative (MIDA-Q) unprecedented registries include 8187 consecutive patients (age 64±17 years, 45% women, follow-up 5.5±3.4 years) with isolated degenerative mitral valve disease diagnosed at tertiary (European/ North-American/ Middle Eastern) centers in whom DMR severity used both integrative and quantitative grading. The MIDA-Q Score ranged from 0 to 15 depending on accumulating risk factors. Long-term survival overall, under medical management and post-mitral surgery was analysed.

Results
By quantitative grading, MR was no/trivial in 1938 (24%), mild in 1423 (17%), moderate in 2027 (25%) and severe in 2799 (34%), with ERO 0.24±24cm2, RVol 37±35mL, and posterior leaflet prolapse in 49%. MIDA-Q Scores stratified in 8 categories were 0 (score 0, n=851), 1 (score 1–2, n=1301), 2 (score 3–4, n=2043), 3 (score 5–6, n=1581), 4 (score 7–8, n=1273), 5 (score 9–10, n=718), 6 (score 11–12, n=331) and 7 (score 13–15, n=89). In the whole MIDA-Q population (n=8187 patients), 5-year survival under medical management with Scores categories 0–1, 2–4, and 5–7 was 96±1%, 73±1%, and 61±3% respectively (P&lt;0.0001). Five-year mortality ranged from 3% with MIDA Q-score 0 to 95% with MIDA Q-score 13–15 (P&lt;0.0001). After mitral surgery, 1-year mortality with Scores categories 0–1, 2–4, and 5–7 was 0%, 1%, and 6% respectively and 5-year post-operative survival was 99±1%, 94±1%, and 82±2% (all P&lt;0.0001). In models including age, sex and all guideline-provided prognostic markers, the EuroScoreII and the MIDA Score without DMR severity, the MIDA-Q Score provided incremental prognostic information (P&lt;0.001).

Conclusion
This unheard international cohort of patients with FMV and prospective mitral severity quantitative grading, enables for the first time the calculation of a MIDA-Q Score, highly determinant of survival after diagnosis of FMV with any degree of DMR, that may be very useful for mitral valve prolapse management.

Funding Acknowledgement
Type of funding sources: Public Institution(s). Main funding source(s): Mayo Fundation Figure 1
Socio-economic variations in the clinical presentation, etiology and outcome of infective endocarditis in the ESC-EORP EURO-ENDO (European Infective Endocarditis) registry: a prospective cohort study
Authors: Sengupta S , Prendergast B , Furnaz S , Ronderos R , Almaghraby A , Asch F M , Blechova K , Zaky H , Dworakowski R , Izumi C , Lancellotti P , Habib G ,   the EURO-ENDO Investigators
Published: October 2021

Abstract

Background
Infective endocarditis (IE) is a life threatening disease associated with high mortality and morbidity worldwide. We sought to determine how socio-economic factors may influence variations in epidemiology, clinical presentation, investigation and management (and their consequence upon clinical outcomes) in a large international multi-centre registry.

Methods
The ESC-EORPEURO-ENDO registry comprises a prospective cohort of 3116 adult patients admitted to 156 hospitals in 40 countries with IE between January 2016 and March 2018. We analysed the complete dataset to assess potentially important determinants of variation according to World Bank economic stratification (high income (Group 1) [73.8%]; upper-middle income (Group 2) [17.1%]; lower-middle income (Group 3)[9.1%]).

Results
Patients in Group 3 were younger (median age [IQR]: Group 1 - 66 [54–75] years; Group 2 - 57 [40–68] years; Group 3 - 33 [26–43] years; p&lt;0.001) with a higher prevalence of smoking, intravenous drug use and human immunodeficiency virus (HIV) infection (all p&lt;0.001). Group 3 patients with IE presented later (median [IQR) days since symptom onset: Group 1 - 12 [3–35]; Group 2 - 20 [6–51]; Group 3 - 31 [12–62]; p&lt;0.001) and were more likely to develop congestive heart failure (13.6%; 11.3%; and 22.6%, respectively; p&lt;0.001), septic shock (8.3%; 11.1%; 13.4%; p=0.007), and persistent fever for greater than 7 days (9.6%; 14.4%; 27.9%; p&lt;0.001) following hospital admission. Surgery was performed less frequently in Group 3 (75.4%, 76.8% and 51.3% in Groups 1, 2 and 3, respectively; p&lt;0.001) and mortality was highest in the poorest countries (14.6%; 23.6% and 23.7%, respectively; p&lt;0.001).

Conclusion
Socio-economic factors influence the clinical profile of patients presenting with IE across the world. Despite being younger, patients from the poorest countries presented with more frequent complications and higher mortality associated with delayed diagnosis and less frequent use of surgery.

Funding Acknowledgement
Type of funding sources: None.
The role of right ventricular-arterial coupling in cardiac amyloidosis: a comparison between subtypes and with other genetic and non-genetic hypertrophic cardiomyopathies and prognostic consequences
Authors: Palmiero G , Monda E , Rubino M , Caiazza M , Vetrano E , Di Fraia F , Lioncino M , Dongiglio F , Carciello G , Manganelli F , Ascione L , Caso P , Limongelli G 
Published: October 2021

Abstract

Background
Right ventricular (RV) dysfunction in cardiomyopathies is a consequence of chronic overload (i.e. aortic stenosis) or direct involvement of systemic disorders (i.e. cardiac amyloidosis, CA). The Tricuspid Annular Plane Systolic Excursion/Systolic Pulmonary Artery Pressure ratio (TAPSE/sPAP) has been recently proposed as a surrogate of RV-arterial coupling (RVAC) in many cardiac disorders.

Purpose
This study aims to compare RVAC between CA subgroups and between CA and other forms of genetic and non-genetic cardiomyopathies with hypertrophic phenotype.

Methods
We enrolled 50 patients with CA (26 pts with AL and 24 pts with wild type ATTR form), 75 patients with LV hypertrophy (LVH) [25 patients with HCM, 25 with hypertensive cardiomyopathy (HypCM), and 25 with aortic stenosis]. We analysed right chambers dimensions and classical and novel parameters of RV function [TAPSE, TAPSE/sPAP, St (S' wave at RV TDI), global (RVGLS) and free-wall (RVFWS) strain].

Results
The ATTR group showed higher right dimensions than AL, without differences in all RV systolic parameters (see Table 1). Compared to the LVH group, CA patients showed no differences in RV dimensions while RV systolic parameters, included the TAPSE/sPAP ratio, were significantly reduced. At ROC curve analysis TAPSE (AUC 0.877; 95% CI: 0.811–0.943; p&lt;0,0001) and TAPSE/sPAP ratio (AUC 0.859; 95% CI: 0.783–0.935; p&lt;0,0001) showed the best ability in discriminating CA among other forms of LVH (cut-off 20,5 mm for TAPSE with a sensibility of 80,5% and specificity of 78,8%; cut-off 0,62 for TAPSE/sPAP ratio with a sensibility of 85,4% and a specificity 81,8%). At Kaplan-Meier estimation, the TAPSE/sPAP ratio showed a significantly reduced survival in the lowest interquartile ranges. Moreover, at multivariate analysis TAPSE/sPAP was the only independent prognostic factor (β −5,644; 95% IC: 0,000–0,522; p&lt;0,027).

Discussion
The RVAC is significantly impaired in CA compared to the LVH group but not between CA subgroups. TAPSE/sPAP proved to be a novel echocardiographic parameter useful in discriminating CA among genetic and non-genetic forms of LVH, and that also show prognostic significance.

Funding Acknowledgement
Type of funding sources: None. Figure 1. K-M for TAPSE/sPAP ratio IQ rangesTable 1
Prognostic significance of cardiac injury in COVID-19 patients with and without coronary artery disease
Authors: Barman H , Atici A , Arabaci H O , Sahin I , Gungor B , Dogan S M 
Published: October 2021

Abstract

Objective
COVID-19 is a disease with high mortality, and risk factors for worse clinical outcome have not been well-defined yet. The aim of this study is to delineate the prognostic importance of presence of concomitant cardiac injury on admission in patients with COVID-19.

Methods
For this multi-center retrospective study, data of consecutive patients who were treated for COVID-19 between March 20 - April 20 2020 were collected. Clinical characteristics, laboratory findings and outcomes data were obtained from electronic medical records. In-hospital clinical outcome was compared between patients with and without cardiac injury.

Results
A total of 607 hospitalized patients with COVID-19 were included in the study; the median age was 62.5±14.3 years, and 334 (55%) were male. Cardiac injury was detected in 150 (24.7%) of patients included in the study. Mortality rate was higher in patients with cardiac injury (42% vs. 8%; p&lt;0.01). The frequency of patients who required intensive care unit (ICU) (72% vs.19%), who developed acute kidney injury (AKI) (14% vs. 1%) and acute respiratory distress syndrome (ARDS) (71% vs. 18%) were also higher in patients with cardiac injury. In multivariate analysis, age, coronary artery disease (CAD), elevated CRP levels, and presence of cardiac injury (OR: 10.58, 95% CI: 2.42–46.27; p&lt;0.001) were found to be independent predictors of mortality. In subgroup analysis, including patients free of history of CAD, presence of cardiac injury on admission also predicted mortality (OR: 2.52, 95% CI: 1.17–5.45; p=0.018).

Conclusion
Cardiac injury on admission is associated with worse clinical outcome and higher mortality risk in COVID-19 patients including patients free of previous CAD diagnosis.

Funding Acknowledgement
Type of funding sources: None.
Alpha-protein kinase 3 (ALPK3) truncating variants cause an autosomal dominant form of hypertrophic cardiomyopathy
Authors: Rocha Lopes L , Hernandez S G , Lorenzini M , Futema M , Chumakova O , Villacorta E , Garcia-Pavia P , Bilbao R , Sandin-Fuentes M , Pinilla J G , Rasmussen T B , Revilla-Marti P , Elliott D , Monserrat L , Elliott P ,   ALPK3 consortium
Published: October 2021

Abstract

Background
More than half of hypertrophic cardiomyopathy (HCM) remains genetically unsolved. ALPK3 truncating variants (ALPK3tv) have been described as a cause of autosomal recessive cardiomyopathy in a small number of paediatric cases, but the pathogenicity in heterozygosity as a possible cause of autosomal dominant HCM is unknown.

Aims
To determine the frequency of heterozygous ALPK3tv in patients with HCM and to confirm their pathogenicity by means of burden testing in independent cohorts, family co-segregation studies, and functional analysis of an allelic series of ALPK3tv using human embryonic stem cell-cardiomyocytes (hESC-CM). Phenotype was compared with a cohort of 1679 genotyped HCM patients.

Methods and results
In a discovery cohort of 770 index patients with HCM, 12 (1.56%) were heterozygous for ALPK3tv, odds ratio (OR) 16.01 (95% confidence interval (95% CI): 7.89 to 29.74, p&lt;8.36e-11), compared to the Genome Aggregation Database (gnomAD) population. In a validation cohort of 2047 HCM probands, 32 (1.56%) carried heterozygous ALPK3tv, OR 16.17 (95% CI: 10.31 to 24.87; p&lt;2.2e-16, compared to gnomAD). Combined logarithm of odds score in 7 families with ALPK3tv was 2.99. In comparison with a large cohort of genotyped HCM patients, the phenotype of 51 HCM patients with ALPK3tv (probands and relatives) was characterised by a higher prevalence of apical/concentric patterns of hypertrophy (60%) compared to both sarcomere-positives or negatives (p&lt;0.001 overall), with the age at diagnosis (56±16ys) and maximum wall thickness (18±4mm) similar to sarcomere-negatives and LV systolic impairment at baseline (6%) and non-sustained ventricular tachycardia (31%) similar to sarcomere-positives. Short PR (10%, p=0.009 overall) and extensive fibrosis&gt;15% of LV segments (49%) were distinctive features. During follow-up (5.3±5.7 years), 4 (9%) patients died of heart failure or had cardiac transplantation (p=0.012 vs sarcomere-negatives and p=0.425 vs sarcomere-positives). Analysis of hESC-CM showed that ALPK3 heterozygotes had phenotypic characteristics of HCM, including increased contractile force and delayed membrane repolarization.

Conclusions
Heterozygous ALPK3tv are pathogenic and segregate with a characteristic HCM phenotype.

Funding Acknowledgement
Type of funding sources: Public Institution(s). Main funding source(s): MRC UK, UCLH BRC
Early onset of heart failure in Japanese ARVC patients with pathogenic desmosomal gene variants
Authors: Sonoda K , Nagase S , Aiba T , Fukuyama M , Kato K , Kusano K , Horie M , Ohno S 
Published: October 2021

Abstract

Background
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy mainly caused by desmosomal gene variants. Although there are a lot of reports regarding to European ARVC patients, the ones in Asian are a few, and the characteristics of Asian ARVC have been still unclear. We have reported that the genetic backgrounds in Japanese ARVC patients were different from those in European ones. In this study, we aimed to examine the phenotype and outcome of Japanese ARVC patients with specific genetic backgrounds.

Methods and results
This study included 104 Japanese ARVC patients who were diagnosed as definite in the 2010 Task Force Criteria for ARVC and received genetic analysis (79 males [76%]; median age at diagnosis, 40 years [IQR 22–53 years].) Fifty-seven patients carried variants in desmosomal genes classified as pathogenic based on ACMG guideline: 30 in DSG2 (29%), 22 in PKP2 (21%), 2 in DSC2 (2%) and 3 in DSP (3%). The median age of diagnosis was significantly younger in the patients with the pathogenic variants than in those without (37 years [IQR 21–49 years] vs. 46 years [IQR 34–58 years], P=0.01). During a median follow-up of 9.3 years (IQR 3.5–20.9 years), 10 patients died and 2 were received heart transplantation. Sixty-two suffered lethal arrhythmic events including cardiopulmonary arrest, ventricular fibrillation, sustained ventricular tachycardia and appropriate shocks by implantable cardioverter defibrillator. Twenty-two were hospitalized for heart failure. There was no difference in these events rate between the two groups. However, survival analysis revealed that patients with pathogenic variants hospitalized for heart failure significantly earlier in their life than those without (P=0.04, log-rank test, Figure 1).

Conclusions
The Japanese ARVC patients with pathogenic variants in desmosomal genes are diagnosed and hospitalize for heart failure at younger age than those without. These findings have not been reported in other ethnics. Our study warns that we should be cautious for not only the prevention of lethal arrhythmic events but also the progress of the heart failure in Japanese ARVC patients with pathogenic variants.

Funding Acknowledgement
Type of funding sources: Foundation. Main funding source(s): Japan Agency for Medical Research and Development Figure 1
Management strategies for atrial fibrillation and flutter in patients with transthyretin cardiac amyloidosis
Authors: Dale Z , Chandrashekar P , Al-Rashdan L , Kim M , Masri A , Nazer B 
Published: October 2021

Abstract

Background
Atrial fibrillation (AF) and flutter (AFL) are common in transthyretin cardiac amyloidosis (ATTR-CM). Ventricular rate control drugs in ATTR-CM are poorly tolerated but data addressing tolerability and efficacy of rhythm control strategies is limited.

Purpose
We report characteristics and outcomes of AF/AFL in a cohort with ATTR-CM.

Methods
A single center observational study of patients seen at our Amyloidosis Center with wild-type or hereditary ATTR-CM diagnosed between 2005–2019. Treatment was prescribed as per treating cardiologists.

Results
Eighty-four patients with ATTR-CM (average age 74±10 years, 94% male) had mean follow-up of 27.6±22.8 months. AF/AFL occurred in 61 patients (73%). Clinically significant rapid ventricular response (RVR) was common as well attempted rate control with AV node blockers (Table 1). However, discontinuation was frequent (80%), often for adverse effects of hypotension (33%), bradycardia (15%), or presyncope/syncope (10%). Rhythm control was initiated in 64%, most often with cardioversion (DCCV) or ablation (Table 2). Post-DCCV recurrence was common (91%) and time to recurrence did not differ with use of anti-arrhythmic drugs (AAD; 5.8 months (IQR 1.9–12.5) vs without AAD 6.2 months (IQR 1.9–12.5) p=0.83). TEE was performed for 33% of DCCV with thrombus seen in 11% of cases – all patients who were not anticoagulated at the time. TEE was otherwise deferred due to known AF/AFL duration &lt;48 hours (13%) or adequate anticoagulation (54%). Ablation was performed in 23% of patients with AFL (all for typical AFL) with 2 patients (14%) having recurrence after mean of 60.9 months. Pulmonary vein isolation for AF was performed in 12% (86% for persistent AF) with 86% recurrence after median of 6.2 months (IQR 5.6–12.3). Most patients (62%) with rhythm control had subjective improvement (≥1 NYHA class or resolved palpitations). Among AAD, amiodarone was most well tolerated with only 8% of patient discontinuing due to side effects. DCCV and ablation resulted in no direct complications although one patient had a perforation of a previously unknown Zenker diverticulum during TEE pre-DCCV.

Conclusions
In our ATTR-CM cohort, AF/AFL was common. Rate control was poorly tolerated and often abandoned. While rhythm control of AF/AFL had a favorable safety profile and successful conversion to sinus rhythm led to symptomatic improvement in a majority of cases, durable success with rhythm control was limited, often requiring multiple therapies. DCCV is only modestly successful and not significant improved with AAD. Ablation was successful in cases of cavo-tricuspid isthmus dependent AFL but had limited success in AF.

Funding Acknowledgement
Type of funding sources: None.