bluuebunny/crossref_metadata_2025_split huggingface dataset:
Prognostic value of left ventricle global constructive work in patients with cardiac amyloidosis
Authors: Geers J , Luchian M L , Motoc A , De Winter J , Roosens B , Cosyns B , Droogmans S 
Published: October 2022

Abstract

Background
Left ventricle (LV) global longitudinal strain (LVGLS) has demonstrated incremental diagnostic and prognostic value in patients with cardiac amyloidosis (CA), however it is sensitive to loading conditions (1). Therefore, myocardial work (MW) analysis emerged as a novel echocardiographic method that incorporates both GLS and afterload for a better assessment of LV performance in several pathologies (2). Nevertheless, data regarding its additional value in patients with CA remains limited.

Purpose
We aimed to evaluate the additional prognostic value of global constructive work (GCW), assessed using MW analysis, in patients with CA.

Method
Seventy-one unselected CA patients were included retrospectively between 2015 and 2021. Patients underwent comprehensive clinical, biochemical and imaging evaluation including two-dimentional (2D) echocardiography with MW analysis. The primary endpoint was defined as all-cause mortality and heart failure requiring hospitalization.

Results
The population (mean age 76,76±11.52 years, 73.2% males) consisted of 53 (74.6%) patients with transthyretin (ATTR), 14 (19.7%) patients with light-chain (AL), and 4 (5.6%) patients with unknown phenotype of CA. The primary endpoint was reached in 37 (52.1%) patients. Patient with the primary endpoint had a significantly lower LV ejection fraction (LVEF) (40.54% vs. 49.31%, p=0.003), LVGLS (−9.26% vs. −11.32%, p=0.017) and GCW (1034.47mmHg% vs. 1424.86 mmHg%, p=0.005) than those without. No significant clinical differences were found between the two groups. Multivariable analysis (Figure 1) showed that LVEF (p=0.003; odds ratio (OR): 0.881; 95% confidence interval (CI): 0.810–0.959), LVGLS (p=0.021; OR: 0.688; 95% CI: 0.502–0.944) and GCW (p=0.016; OR: 0.995; 95% CI: 0.991–0.999) were independent predictors for the primary outcome. The model including GCW had the best discriminative ability to predict the primary endpoint (C-index = 0.900; 95% CI: 0.781–1.000, p=0.001). A GCW less than 1443mmHg% was able to predict the primary endpoint with a sensitivity of 94.1% and a specificity of 64.3% (Figure 2; area under the curve (AUC): 0.771 (95% CI: 0.581–0.961; p=0.005).

Conclusion
In patients with CA, a decrease in GCW as a reflection of an impaired systolic performance of the LV was independently associated with an increase in heart failure hospitalization and all-cause mortality. Further prospective studies are warranted in order to confirm the potential role of GCW to improve the prognosis of CA patients.

Funding Acknowledgement
Type of funding sources: None.
Management of fulminant myocarditis: peripheral venoarterial extracorporeal membranous oxygenation and associated complications
Authors: Costa C , Ferraz A L , Ferreira A R , Roncon-Albuquerque R 
Published: October 2022

Abstract

Background
Fulminant Myocarditis (FM) leads to cardiogenic shock with multi-organic dysfunction, being peripheral venoarterial extracorporeal membranous oxygenation (VA-ECMO) a rescue technique. Despite growing experience, it is still related with several complications. We aim to appraise the management of patients with FM and the adverse events associated to VA-ECMO.

Methods
We conducted a retrospective study in a ECMO centre in Portugal, which included 15 patients diagnosed with FM and managed on VA-ECMO from 2008 to 2018. Hemorrhage or infection on cardiac device site, cardiac tamponade, limb ischemia, ischemic stroke, cerebral hemorrhage, severe hemolysis and accidental decannulation were considered major complications.

Results
For sample characteristics and resumed complications, see Table. Inotropic and mechanical ventilation support were transversal to all, with only one patient extubated before decannulation. Six patients received renal replacement therapy, whom half recovered renal function and the remaining died. VA-ECMO operated on average for 8,5 days (range, 1 to 20 days). 3 patients had concomitant Impella heart pump and one of them had a third assistance device with Intra-Aortic Ballon pump.
Major complications were observed in 9 patients (60%), being ischemic stroke the most common. Limb ischemia, observed in 5 patients, related with longer duration of VA-ECMO support (limb ischemia vs non-limb ischemia; 12,8±4,9 vs. 6,8±6,5 days). Two patients presented hemorrhage on cardiac assistance device site, and 1 patient had a cardiac tamponade, all with fatal outcome. Infection on cardiac site, cerebral hemorrhage, severe hemolysis and accidental decannulation were not observed.

Conclusion
The most common complications were ischemic stroke and limb ischemia, both not apparently related with mortality. Differently, hemorrhage on cardiac site and cardiac tamponade were less frequent but presented ominous outcome. Overall, adverse events can be serious and hinder recovery and discharge.

Funding Acknowledgement
Type of funding sources: None.
SARS-CoV-2 vaccination and myocarditis in a highly vaccinated New Zealand population
Authors: Greer C , Daly M , Troughton R , Adamson P D 
Published: October 2022

Abstract

Background
A higher incidence of myocarditis has been reported in those who have recently received mRNA SARS – CoV-2 vaccination.1 Canterbury District Health Board (CDHB) serves 578,290 people, including 441,852 adults, with one large tertiary referral hospital offering specialist cardiology services. In 2021 97% of eligible adults received at least one dose and 92% two doses of the BNT162b2 mRNA vaccine (Pfizer-BioNTech). During this time only 21 community cases of COVID-19 infection were reported. We investigated the incidence of myocarditis during the BNT162b2 mRNA vaccine rollout in comparison to the preceding 5 years assuming a stable population size.

Methods
All adult patients admitted to our hospital who received a diagnostic code of acute myocarditis (ICD10 codes I40, I41 and I51.4) during admission between 2016 and 2021 were included. Demographics and peak troponin concentration (hsTnI) were recorded. Vaccine-associated myocarditis was defined as that leading to admitted within 28 days of BNT162b2 vaccination. Myocarditis-associated mortality was defined as death occurring within 28 days of hospital admission. Incidence of myocarditis before and during COVID-19 vaccination was tested using ANOVA.

Results
Between 2016 and 2020 there were 178 total hospital admissions (annualised mean 35.6 [SD6.3] range 28–44) with myocarditis. The mean age was 47.8 [SD15.9] years, 38% were women, and median peak hsTnI 641 (IQR 95.25–8526) ng/L. One patient died within 28 days of admission. In 2021 there were 43 myocarditis admissions, mean age 49.7 [SD18] years, 42% women, with a median hsTnI 355 (IQR 106.5–1876.5) ng/L. Nine of these admissions were within 28 days of vaccination. They were 78% female, mean age 52.6 [SD24.8] years, median peak hsTnI 179 (IQR 52–528) ng/L. One patient died during admission. There was no variance in annual incidence of myocarditis during vaccine rollout (p=0.342).

Conclusion
In a highly vaccinated adult population largely free of COVID-19 infection there were few cases of myocarditis within 28 days of vaccination and no increase in incidence overall compared to the preceding 5 years.

Funding Acknowledgement
Type of funding sources: Foundation. Main funding source(s): Heart Foundation of New Zealand grant to C Greer
HCM-AF South Italy score for prediction of new-onset atrial fibrillation in patients with hypertrophic cardiomyopathy: data from a derivation and validation italian cohorts
Authors: Todde G , Monda E , Canciello G , Lioncino M , Lombardi R , Rubino M , Borrelli F , Caiazza M , Paoletta D , De Simone G , Pacileo G , Esposito G , Limongelli G , Losi M A 
Published: October 2022

Abstract

Background
Only one score for prediction of new-onset atrial fibrillation (AF) in patients with hypertrophic cardiomyopathy (HCM) has been developed in North America (HCM-AF North America score).

Purpose
To develop a new score (HCM-AF South Italy score) in an Italian derivation cohort and to test the new score with that from North America, in an Italian validation cohort.

Methods
A cohort of 228 patients with HCM and without history of AF, followed at referral center 1 for HCM, was used for model development. A cohort of 226 patients without history of AF, followed at referral center 2, was used for external validation. During follow-up (IQR 3–13 years, median 8 years), 59 (26%) patients developed new-onset AF. Data in the development cohort generated the HCM-AF South Italy score, a point score to predict AF probability: left atrial dimension &gt;49 mm (+5 points), age at clinical evaluation &lt;55 years (+2 points), the use of angiotensin-converting enzyme (ACE) or inhibitor or angiotensin receptor blocker (ARB) at initial evaluation (+2 points), low left ventricular (LV) ejection fraction (&lt;50%) (+2 points), LV outflow tract obstruction (LVOTO) &gt;25 mmHg (+2 points), NYHA class &gt;1 (+1 point).

Results
Patients from the validation cohort were older, had lower prevalence of HCM family history, lower systolic and diastolic blood pressure, taken fewer drugs like beta-blockers, ACE or ARB, calcium-channel blockers, and antiarrhythmic drugs and had lower LVOTO than patients from the derivation cohort, (overall p&lt;0.05). During follow-up (IQR 1–10 years, median 5 years), 25 (11%) patients developed AF. ROC curve analysis demonstrated that HCM-AF South Italy score was superior to HCM-AF North America score (Figure). A HCM- AF South Italy score &gt;4 identified patients at risk of AF with a sensitivity of 48%, specificity of 76%, PPV of 20% and NPV of 92%.

Conclusion
Although derived and tested in little and quite different populations, the performance of the HCM-AF South Italy score was superior to that of the score developed in North America.

Funding Acknowledgement
Type of funding sources: None.
Heterogeneous mechanisms of pulmonary congestion in hypertrophic cardiomyopathy unmasked by comprehensive exercise stress echocardiography
Authors: Palinkas E D , Re F , Peteiro J , Tesic M , Palinkas A , Torres M A R , Djordjevic Dikic A , Beleslin B , Van De Heyning C M , D'Alfonso M G , Mori F , Ciampi Q , Sepp R , Olivotto I , Picano E 
Published: October 2022

Abstract

Background
B-lines detected by lung ultrasound (LUS) during exercise stress echocardiography (ESE), indicating pulmonary congestion, are present in 1 out of 3 patients with hypertrophic cardiomyopathy (HCM).

Aim
To assess the functional and anatomical correlates of exercise B-lines in HCM.

Methods
We enrolled 191 HCM patients (age 53±15 years, 123 males) consecutively referred for ESE (treadmill in 74, bicycle in 117 patients) in 11 quality-controlled centers from 8 countries. ESE assessment at rest and peak stress included: left ventricular (LV) outflow tract gradient, left atrial (LAVi) and LV end-diastolic volume index (EDVi), mitral regurgitation (MR, score from 0 to 3); E/e'; systolic pulmonary arterial pressure (SPAP) and LV force (LV outflow tract gradient+systolic blood pressure/LV end-systolic volume). B-lines at rest and at peak exercise were assessed by LUS with the 4-site simplified scan. B-lines positivity was considered if the sum of detected B-lines was ≥2.

Results
LUS was feasible in all subjects. B-lines were present in 55 (29%) patients during stress. When compared to patients without stress B-lines (n=136), patients with B-lines (n=55) at peak exercise had lower peak EDVi (43±17 vs 52±18 ml/m2, p=0.003) higher peak E/e' (16±6 vs 12±5, p&lt;0.001), increase in MR (34 vs 12%, p=0.001), greater stress LAVi (43±14 vs 37±14 ml/m2, p=0.003) and stress SPAP (56±18 vs 40±12 mm Hg p&lt;0.0001): see Figure. Among baseline parameters, the number of B-lines (OR: 7.53, 95% CI 1.21–46.72 p=0.03), LAVi (OR: 1.05, 95% CI 1.00–1.09 p=0.04), and LV force (OR: 1.36, 95% CI 1.04–1.79 p=0.03) were the independent predictors of exercise pulmonary congestion.

Conclusion
HCM patients with pulmonary congestion on exercise show different, and not mutually exclusive mechanisms of diastolic dysfunction and worsening mitral regurgitation. These different hemodynamic mechanisms may require personalized therapeutic actions beyond a pulmonary decongestion therapy with diuretics.

Funding Acknowledgement
Type of funding sources: None.
Sudden cardiac death during exercise in young individuals with hypertrophic cardiomyopathy
Authors: Bhatia R , Westaby J , Behr E , Papadakis M , Sharma S , Finocchiaro G , Sheppard M 
Published: October 2022

Abstract

Background
Sudden cardiac death (SCD) in young individuals and athletes is generally caused by hereditary cardiac conditions, including cardiomyopathies such as hypertrophic cardiomyopathy (HCM). Although historically HCM has been reported as the predominant cause of SCD in young athletes, it is unclear as to what degree exercise is a trigger for possible fatal arrhythmias.

Aim
We aimed to report on the circumstances of SCD in a cohort of young individuals aged ≥10 and &lt;30 whose autopsy was consistent with HCM.

Methods
We reviewed 6860 consecutive cases of SCD referred to our specialist cardiac pathology centre 1994 and 2020. SCD was defined as death from a cardiovascular cause within 12 hours of apparent well-being. All cases underwent detailed autopsy evaluation of the heart, including histological analysis, by expert cardiac pathologists. A minimum of 10 blocks of tissue were taken for histological analysis. HCM was defined by the presence of increased heart weight or increased wall thickness and significant myocyte disarray at histological examination.

Results
Of the total cases of SCD, 264 (4%) were due to HCM. Our cohort of young decedents comprised of 66 individuals (average age 21±5 years, males 76%). For the majority (n=52, 79%) SCD was the first manifestation of HCM. The average heart weight was 507±152 grams and left ventricular (LV) fibrosis was found in 28 (42%) cases (Figure 1A). Death was more common between 16 and 20 years of age (n=24) (Figure 1B). Death occurred during exertion in 25 (38%) individuals and at rest or during daily activities in the remaining 41 (62%), including 5 individuals who died during sleep. Male sex was more represented among decedents who died during exertion (88% compared with 68% in the group that died at rest, p=0.07); LV fibrosis was more commonly observed in individuals who died during exertion (56% compared with 34% in the group who died at rest, p=0.08). Younger individuals between 10–15 years of age died mostly during exercise (80%), in other age groups death occurred mainly at rest (33% in age group 16–20 years, 30% in age group 21–25 years, 33% in age group 26–30 years) (Figure 1B).

Conclusions
We observed a high age-related variability in terms of circumstances of death. In the context of HCM, our findings suggest that individuals aged 10–15 years are the most vulnerable in terms of exercise-related-SCD. This exemplifies the importance of preventative cardiac screening in young individuals who might be harbouring quiescent cardiac conditions associated with young SCD.

Funding Acknowledgement
Type of funding sources: Other. Main funding source(s): Acknowledgements: We thank the charitable organisation, Cardiac Risk in the Young (CRY) who fund and support the CRY Cardiovascular Pathology Unit and CRY database.
New-onset atrial fibrillation diagnosed by cardiac implantable devices in patients with hypertrophic cardiomyopathy: a systematic review and meta-analysis
Authors: Hurtado V , Fernandes A , Silva M M C A , Fernandes G C , Cardoso R , Davila A 
Published: October 2022

Abstract

Background
Atrial fibrillation (AF) in patients with hypertrophic cardiomyopathy (HCM) is associated with high thromboembolic risk, morbidity, and mortality.

Purpose
We aimed to study the role of cardiac implantable devices (CID) for AF diagnosis in patients with HCM without prior AF history.

Methods
A comprehensive systematic review and meta-analysis was conducted using Pubmed, Embase, and ClinicalTrials.gov through November 2021 for studies reporting incidence of new-onset AF detected by CID including implantable cardioverter defibrillators, pacemakers, cardiac resynchronization therapy, and loop recorder devices in HCM patients. We used a Freeman-Tukey transformation to calculate the weighted summary proportion of the incidence of AF and stroke and pooled odds ratios (OR) with 95% confidence intervals (CI) were calculated using a random-effects model.

Results
Eight cohort studies were included, totalizing 910 patients with HCM and CID without baseline AF. Mean age was 54 years and 21% were female. Mean left ventricular wall thickness was 22.6 mm. Mean follow-up was 3.1 years. New-onset AF was detected in 27.6% (95% CI 18.7–37.5) of patients (Figure A) and 84% of the episodes were subclinical. Stroke occurred in 4.7% (95% CI 1.8–9.0) of patients without baseline AF (Figure B). There was no significant difference in the unadjusted risk of stroke between patients with newly diagnosed AF vs no AF detected at the end of follow-up (9.4% vs 4.7%, OR 1.93, 95% CI 0.83–4.48, p=0.13).

Conclusion
There is a high incidence of subclinical new-onset AF in patients with HCM, supporting the use of CID for early detection of AF in this population.

Funding Acknowledgement
Type of funding sources: None.
Predictors of left ventricular dysfunction in hypertrophic cardiomyopathy: results from a nationwide registry
Authors: Brandao M , Goncalves Almeida J , Fonseca P , Faria R , Sousa O , Fonseca C , Fontes-Carvalho R 
Published: October 2022

Abstract

Introduction
Progression of hypertrophic cardiomyopathy (HCM) with incident left ventricular (LV) dysfunction (HCM-LVSD) is associated with poor prognosis and advanced heart failure (HF). Prevalence ranges from 5–10% in previous studies. Identification of predictors of adverse remodeling may improve risk stratification and prognostication in HCM.

Purpose
To identify predictors of HCM-LVSD in a nationwide cohort of HCM patients (pts).

Methods
Retrospective study including all HCM pts enrolled in a European country-based nationwide registry. HCM-LVSD group included pts with LV ejection fraction (LVEF) ≤50% at baseline and pts who developed LV dysfunction/dilated phenotype during follow-up. Multivariate logistic regression was performed to identify predictors of HCM-LVSD.

Results
1042 HCM patients (57.8% male, mean age at diagnosis 52 years) were included, of whom 81 (8%) belonged to the HCM-LVSD group. HCM-LVSD pts were mostly male (60.5%) and tended to be older at the time of diagnosis than those without LVSD (55 vs 52 years, p=0.054). HCM-LVSD pts were more often symptomatic at the index visit (84.1% vs 65.2%, p&lt;0.001), with more functional impairment (New York Heart Association class III–IV: 18.5% vs 9.2%, p=0.021). Atrial fibrillation (21.3% vs 8.6%, p&lt;0.001) and intraventricular conduction disturbances (28.6% vs 14.4%, p=0.002) were more prevalent in HCM-LVSD pts. HCM-LVSD pts had higher baseline left atrium (LA) volumes (52 vs 39 ml, p=0.001), lower LVEF (50 vs 67%, p&lt;0.001) and higher rates of mitral regurgitation (79.0% vs 65.1%, p=0.011). Prevalence of obstructive HCM was lower in the HCM-LSVD group (25.3% vs 40.9%, p=0.007). Presence of late gadolinium enhancement (92.6% vs 74.6%, p=0.035) was more common in pts with LVSD. Baseline N-terminal pro–B-type natriuretic peptide was higher in HCM-LVSD (3839 vs 1281 pg/ml, p=0.027). There were no differences in the number and type of genetic variants between groups. In HCM-LVSD pts, implantation of cardioverter-defibrillators for secondary prevention was more frequent (28.6% vs 6.4%, p=0.002), as was the use of pacemaker (16.7% vs 7.0%, p=0.002). During a mean follow-up of 5.3±6.1 years, hospitalization for HF (50.0% vs 11.3%) and all-cause mortality (12.3% vs 2.9%, p&lt;0.001) were more frequent in HCM-LVSD group.
After multivariate analysis, higher LA volume (odds ratio [OR] 1.03, 95% confidence interval [CI] 1.01–1.05, p=0.003) and nonobstructive HCM (OR 2.74, 95% CI 1.03–7.27, p=0.043) were independent predictors of HCM-LVSD.

Conclusions
In this large nationwide cohort of HCM pts, prevalence of LVSD was 8%, in line with existing literature. In this cohort, larger LA volumes and nonobstructive HCM predicted progression to HCM-LVSD.

Funding Acknowledgement
Type of funding sources: None.
Incidence of stroke in patients with hypertrophic cardiomyopathy in stable sinus rhythm during long-term monitoring
Authors: Fumagalli C , Bonanni F , Beltrami M , Ruggiero R , Zocchi C , Tassetti L , Maurizi N , Zampieri M , Lovero F , Di Bari M , Marchionni N , Pieragnoli P , Cappelli F , Fumagalli S , Olivotto I 
Published: October 2022

Abstract

Background
Patients with hypertrophic cardiomyopathy (HCM) are at increased risk of atrial fibrillation (AF) and stroke, especially at an advanced disease stage. To date, however, the incidence and factors associated with cardioembolic events in HCM patients without AF remain unresolved.

Purpose
To determine the incidence of stroke in HCM patients in whom cardiac rhythm was monitored with a cardiac implantable electronic device (CIED). The association of stroke with left atrial (LA) enlargement was also examined.

Methods
Retrospective cohort study in an outpatient clinic in a tertiary HCM Referral Center.
All consecutive patients diagnosed with HCM and referred for CIED implantation with &gt;16 years at diagnosis and &gt;1 year follow-up post CIED implantation were reviewed. Severe LA dilatation was defined as a LA dimension (LAD) of &gt;48mm at echocardiogram. Based on CIED monitoring, patients were classified as: Pre-existing AF (diagnosed with AF prior to CIED); De novo AF (diagnosed with AF after CIED implantation); Sinus Rhythm (SR): no episodes of AF. Incidence of stroke after CIED implantation was the primary outcome.

Results
A total of 185 patients (57% men, age: 54±17 years) were implanted with a CIED and were included. Pre-existing AF was present in 72 (36%) patients and de novo AF in 24 (13%); whereas 89 (48%) remained in SR. After 5 [2–9] years, stroke was reported in 19 (10.3%) patients: 7 occurred in patients with pre-existing AF (1.1%/year), 3 in patients with de novo AF (2.2%/year), and 9 in patients with SR (2.3%/year). No difference was captured by CHA2DS2-VASc score among rhythm categories. Patients with AF had larger LAD at baseline. Among patients in SR, those with a LAD&gt;48mm had the greatest risk of stroke (4.8%/year vs 0.5%/year, p&lt;0.01; Hazard Ratio [HR]: 8.56, 95% C.I. 2.03–36.15). At Cox multivariable regression analysis, LA (HR: 1.104, 95%C.I. 1.039–1.173, p=0.001) and AF (HR: 0.310, 95% C.I. 0.102–0.939, p=0.038) were associated with incident stroke.

Conclusions
In HCM patients with CIED long-term monitoring and no prior history of AF, stroke rates were similar in those with de novo AF or stable sinus rhythm. CHA2DS2-VASc considerably underestimated risk, whereas severe LA dilatation was a powerful predictor of risk, irrespective of AF.

Funding Acknowledgement
Type of funding sources: None.