bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical, functional and prognostic correlates of excess left ventricular force in hypertrophic cardiomyopathy
Authors: Palinkas E D , Marchi A , Milazzo A , Tassetti L , Zampieri M , D'Alfonso M G , Mori F , Palinkas A , Ciampi Q , Sepp R , Olivotto I , Picano E 
Published: October 2022

Abstract

Background
Excess force generation during myocardial contraction represents a cardinal feature of hypertrophic cardiomyopathy (HCM).

Purpose
To evaluate the anatomical, functional and prognostic correlates of left ventricular (LV) force in HCM.

Methods
We prospectively recruited a consecutive sample of 408 HCM patients with LV ejection fraction (EF) &gt;50%, referred for baseline transthoracic echocardiography in 2 primary HCM centers in Hungary and Italy between 1999 and 2021. LV force was calculated as LV outflow tract gradient+systolic blood pressure/LV end-systolic volume. Patients were followed for a median of 107 months (IQ range, 58–158 months), the study endpoint was all-cause mortality.

Results
Mean LV force was 6.0±4.6 mm Hg/ml. Receiver-operating characteristic analysis identified 7.5 mm Hg/ml the best cut-off value to predict mortality. LV force &gt;7.5 mm Hg/ml was present in 86 patients (21%), more frequently in women (58 vs 27%, p&lt;0.0001), more often in patients with diabetes (16 vs 6%, p=0.003), beta-blocker (81 vs 58%, p&lt;0.0001), calcium channel-blocker (19 vs 9%, p=0.012) and diuretic therapy (19 vs 8%, p=0.004), compared to patients with LV force ≤7.5 mm Hg/ml. Patients with excess LV force had more advanced NYHA class (1.8±0.7 vs 1.4±0.7, p=0.0001), greater left atrial diameter [(LAd), 46.7±6.6 vs 43.0±7.2 mm, &lt;0.0001], LV maximal wall thickness (23.2±5.5 vs 21.6±5.5 mm p=0.01), LV EF (74.6±6.4 vs 67.0±6.8%, &lt;0.0001), mitral regurgitation grade (1.2±0.7 vs 0.7±0.7, p&lt;0.0001) and E/e' (10.3±5.0 vs 7.8±5.0, p=0.005). During follow-up 43 deaths occurred. All-cause death was more frequent in patients with excess LV force (21 vs 8%, p&lt;0.0001). At multivariable Cox regression analysis, excess LV force was an independent predictor of mortality (HR 2.9, 95% CI 1.14–7.26, p=0.025) independent of age (HR 1.03, 95% CI 1.00–1.05, p=0.022) and LAd (HR 1.07, 95% CI 1.02–1.14, p=0.005).

Conclusion
LV force with a threshold of 7.5 mm Hg/ml, independently predicts adverse outcome in patients with HCM and preserved systolic function. Excess LV force generation is associated with female sex, diabetes, NYHA class, medications, LAd, LV wall thickness, EF, mitral regurgitation grade and E/e'.

Funding Acknowledgement
Type of funding sources: None.
3 year outcomes of permanent pacemaker implantation after alcoholic septal ablation
Authors: Grazina A , Ferreira V , Cardoso I , Garcia Bras P , Viegas J M , Aguiar Rosa S , Fiarresga A , Ramos R , Castelo A , Teixeira A R , Teixeira B , Jacinto S , Martins Oliveira M , Cacela D , Cruz Ferreira R 
Published: October 2022

Abstract

Introduction
Patients with hypertrophic obstructive cardiomyopathy (HOCM) that remain symptomatic despite optimized medical therapy are often submitted to alcohol septal ablation (ASA). One of the most frequent complications is the complete hear block (CHB), requiring permanent pacemaker (PPM) in variable rates, up to 20% of the patients. The long-term impact of PPM implantation in these patients remains unclear.

Objectives
This study aims to evaluate the long-term clinical outcomes in patients who implant PPM after ASA.

Methods
In a tertiary center, patients who underwent ASA were consecutively enrolled prospectively. Patients with previous PPM or implantable cardio-defibrillator were from this analysis. The groups with and without PPM implantation after ASA were compared regarding baseline characteristics, procedure data and 3-year primary and secondary endpoints.

Results
Between 2009 and 2020, 109 patients underwent ASA. 97 patients were included in this analysis (68% female, mean age 65.2 years-old). 16 patients (16.5%) required PPM implantation for CHB. In those, no vascular access, pacemaker pocket or pulmonary parenchyma complications were noted. The baseline characteristics regarding comorbidities, symptoms, echocardiographic and electrocardiographic findings were identical in the two groups, with statistically significant differences in the mean age (70.6y/o in the PPM group versus 64.1y/o) and in the beta-blocker therapy rates previously to the intervention (56% in the PPM group versus 84%). Procedure-related data showed higher creatine kinase (CK) peaks in the PPM group (1692U/L versus 1243U/L, p0.05), without significant differences in the alcohol dose (2.1ml in both groups, p0.33). At 3 years after ASA procedure, the primary endpoint (composite of all-cause mortality and all-cause re-hospitalization) showed a statistical tendency to a lower event rate in the group who implanted PPM (p-value 0.097, Graphic 1). The secondary endpoint (composite of all-cause mortality and cardiac cause re-hospitalization) did not show any significant statistical difference between the two groups (p-value 0.216, Graphic 2).

Conclusions
The long-term endpoint analysis suggests that the outcomes in patients who implant PPM after ASA are non-inferior to those who do not, with a tendency to a lower rate of the endpoint composite of all-cause mortality and all cause re-hospitalizations.

Funding Acknowledgement
Type of funding sources: None.
Change in left atrial function and volume predicts heart failure with preserved and reduced ejection fraction: the multi-ethnic study of atherosclerosis
Authors: Lim D , Varadarajan V , Quinaglia T , Pezel T , Wu C , Noda C , Heckbert S , Bluemke D , Ambale-Venkatesh B , Lima J 
Published: October 2022

Abstract

Aims
This study used cardiac magnetic resonance (CMR) imaging to investigate the relationship between longitudinal change in left atrial (LA) function and incident heart failure (HF), in a multi-ethnic population free of known cardiovascular disease at baseline.

Methods and results
In the Multi-Ethnic Study of Atherosclerosis (MESA), 2470 participants (60±9 years, 47% males), free at baseline of clinically recognized cardiovascular disease, had LA volume and function assessed with CMR imaging at baseline (2000–02) and 9.4±0.6 years later. Seventy three (3%) participants developed incident HF over 7.1±2.1 years; of these, 39 participants had preserved ejection fraction (HFpEF) and 34 participants had reduced ejection fraction (HFrEF). An annual decrease of 1-SD unit in peak LA strain (ΔLASmax) was most strongly associated with the risk of HFpEF (subdistribution HR=2.56, 95% confidence interval (CI) [1.34–4.90], P=0.004) and improved model reclassification and discrimination in predicting HFpEF (c-statistic=0.84, 95% CI [0.79–0.90]; NRI=0.34, P=0.01; IDI=0.02, P=0.02; x2=4.25, P=0.89) adjusting for event-specific risk factors and baseline LA parameters. An annual decrease of 1ml/m2 of pre-atrial LA volume index (ΔLAVipreA) was most strongly associated with the risk of HFrEF (subdistribution HR=1.88, 95% CI [1.44–2.45], P&lt;0.001) and improved model reclassification and discrimination in predicting HFrEF (c-statistic=0.81, 95% CI (0.72–0.90); NRI=0.31, P=0.03; IDI=0.01, P=0.50; x2=15.4, P=0.08), adjusting for event-specific risk factors and baseline LA measures (Table 1).

Conclusion
In this multi-ethnic population free of clinical cardiovascular disease at baseline, ΔLASmax and ΔLAVipreA were associated with, and incrementally predictive of HFpEF and HFrEF respectively, after adjusting for event-specific risk factors and baseline LA measures.

Disclaimer
The views expressed in this abstract are those of the authors and do not necessarily represent the views of the National Heart, Lung, and Blood Institute; the National Institutes of Health; or the U.S. Department of Health and Human Services.

Funding Acknowledgement
Type of funding sources: Public Institution(s). Main funding source(s): National Heart, Lung and Blood InstituteNational Center for Advancing Translational Sciences
Representativeness of EXPLORER-HCM trial and prevalence of eligibility criteria for Mavacamten in a nationwide cohort
Authors: Brandao M , Goncalves Almeida J , Fonseca P , Faria R , Sousa O , Fonseca C , Fontes-Carvalho R 
Published: October 2022

Abstract

Introduction
The EXPLORER-HCM trial evaluated the safety and efficacy of Mavacamten, a cardiac myosin inhibitor, in symptomatic obstructive hypertrophic cardiomyopathy (HCM). Mavacamten improved symptoms, exercise capacity and left ventricular outflow tract (LVOT) obstruction. However, the proportion of patients eligible for this therapy in real practice remains unclear.

Purpose
To determine the prevalence of eligibility criteria for Mavacamten and to describe the profile of patients complying with EXPLORER-HCM criteria in a nationwide cohort.

Methods
Cross-sectional study including all HCM patients enrolled in a European country-based nationwide registry. Presence of EXPLORER-HCM inclusion [≥18 years, obstructive HCM (LVOT gradient ≥50 mmHg), left ventricular ejection fraction (LVEF) ≥55%, New York Heart Association (NYHA) class II–III] and exclusion criteria [syncope or sustained ventricular tachycardia with exercise] were analyzed.

Results
Of 1042 patients included in this analysis (57.8% male, mean age at diagnosis 52 years, 39.6% obstructive HCM), 45 met the EXPLORER-HCM inclusion criteria and, after applying exclusion criteria, 30 patients were eligible for therapy with Mavacamten.
Eligible patients were all Caucasian, mostly female (63.3%), with a mean age at diagnosis of 58±14 years. All patients presented with heart failure symptoms, and were mostly in NYHA class II (83.3%); 4 patients (13.3%) presented with atrial fibrillation. In the baseline echocardiogram, mean LVEF was 68±9%; mean interventricular septum thickness was 20±4 mm; most patients presented with systolic anterior motion of the mitral valve (96.7%) and mitral regurgitation (96.7%). In cardiac magnetic resonance imaging evaluation, maximal wall thickness was 22±4 mm and 76.9% had late gadolinium enhancement. A pathogenic or likely pathogenic HCM gene variant was identified in 5 patients (MYH7 and MYBPC3 gene mutations).
In this group, 86.2% of patients were treated with beta-blockers and 34.5% with calcium channel antagonists. Only 1 patient was on Disopyramide. Septal reduction techniques were used in 43.3% of patients: alcohol septal ablation and septal myectomy were performed in 7 (23.3%) and 6 (20.0%) patients, respectively. Cardioverter-defibrillators were implanted in 2 patients for primary prevention of sudden cardiac death, and 6 patients received a pacemaker for bradycardia indications.

Conclusion
Specific therapies remain an unmet need in HCM. In this large nationwide cohort, only 2.9% of patients were eligible for therapy with Mavacamten according to EXPLORER-HCM criteria. Among patients complying with the study criteria, invasive septal reduction therapies were offered to a high proportion of patients. Further real-world data are warranted to estimate the proportion of HCM patients that will benefit from Mavacamten.

Funding Acknowledgement
Type of funding sources: None.
Temporal trends, characteristics and comorbidities of patients with hypertrophic cardiomyopathy in Denmark from 2005 to 2018: a nationwide cohort study
Authors: Zoerner C R , Pallisgaard J L , Schjerning A M , Toennesen J , Jensen M K , Gislason G , Hansen M L 
Published: October 2022

Abstract

Background
The majority of patients with Hypertrophic cardiomyopathy (HCM) are mildly symptomatic or unaware of their condition, but some develop serious complications such as heart failure, atrial fibrillation (AF) as well as sudden cardiac death. Previous studies have suggested that HCM detection-rates in women are significantly lower than in men. This leads to diagnosis often being delayed to later stages of the disease in women, where symptoms are more severe. Further characterization of HCM patients to improve early detection of adverse outcomes and warning signs might improve long-term outcomes.

Purpose
To describe the characteristics of Danish patients diagnosed with HCM between 2005–2018 and determine trends and changes in these factors over time.

Methods
All patients aged 16 years or older with a diagnosis of HCM between the 1st of January 2005 and the 31st of December 2018 were identified in Danish nationwide administrative registers and included in the study.
Time trends were calculated, and differences analyzed using the Cochran-Armitage trend test and linear regression.

Results
A total of 3856 patients were diagnosed with HCM in the study period and included in the study. The median age at diagnosis was 68 years (IQR 56 and 78 years), 53% were male, and 44% were diagnosed with obstructive HCM, while the number of patients diagnosed with HCM each year overall increased. At the time of diagnosis, 22,3% patients were previously diagnosed with ischemic heart disease, 17% with AF, 7,5% with ischemic stroke, 13,6% with heart failure. Median age and gender distribution remained stable over time, while the proportion of obstructive HCM among the newly diagnosed decreased (p = &lt;0.001) (Figure1). During the study period, there was a significant decrease in the prevalence of heart failure (p = &lt;0.001), ischemic heart disease (p = &lt;0.001), and chronic obstructive pulmonary syndrome (p = &lt;0.001), while the prevalence of AF, hypertension and ischemic stroke remained stable (Figure 2).

Conclusion
Despite previous studies describing a gender gap in patients diagnosed with HCM, gender distribution was near equal in this cohort. The number of patients diagnosed with non-obstructive HCM is increasing and the prevalence of comorbidities such as heart failure, ischemic heart disease, and COPD decreased over time.
Whether these findings are the result of improved early detection of HCM warrants further research and examination.

Funding Acknowledgement
Type of funding sources: Public hospital(s). Main funding source(s): Herlev-Gentofte Hospital, Denmark
Outcomes in hypertrophic obstructive cardiomyopathy patients undergoing non-cardiac surgery-a nationwide analysis
Authors: Victor V , Abumoawad A , Ibrahim F , Barssoum K 
Published: October 2022

Abstract

Introduction
Patients with hypertrophic obstructive cardiomyopathy (HOCM) have a unique pathophysiological profile that increases their chances of having adverse events in the postoperative period following surgical procedures. Owing to the rarity of the disease, few studies are available for assessing readmission risk in HOCM patients undergoing noncardiac surgical procedures.

Purpose
Our study aimed to assess 30-day readmission rates and causes for readmissions among HOCM patients who had undergone noncardiac surgeries in hospitals across the United States (US).

Methods
We used appropriate International Classification of Diseases Code, 10th Revision Clinical Modification (ICD-10-CM) codes to identify patients who had been admitted for non-cardiac surgeries between January 2016 and December 2019 based on data from the National Readmissions Database (NRD). The NRD is a publicly available all-payer in-patient database sponsored by the Agency for Healthcare Research and Quality, containing discharge data from approximately 18 million discharges each year, accounting for 61.8% of the total US resident population. Patients were subdivided into two sub-groups based on the presence or absence of HOCM. 30-day readmission rates and causes of readmissions were compared between both groups. STATA version 17 (College Station,TX: StataCorp LLC) was used for statistical analyses.

Results
A total of 5,497,134 patients out of whom 2,089 had HOCM were identified and included for analysis. Patients with HOCM had statistically significant higher rates of readmissions for central nervous system related complications such as stroke or transient ischemic attack (TIA), cardiogenic shock, acute heart failure, unspecified iatrogenic cardiac complications, acute kidney injury, sepsis in addition to higher rates of post op respiratory failure, acute myocardial infarction and major adverse cardiovascular and cerebrovascular events (MACCE) during the index admission when compared to the other subgroup.

Conclusion
Our study, despite being retrospective in nature, is one of the largest to date assessing readmission risk in HOCM patients undergoing noncardiac surgeries. Similar to previous studies, HOCM patients have higher rates of readmissions and MACCE in the 30-day period following noncardiac surgical procedures. In light of these findings, shared medical decision making and appropriate counseling regarding complications in the postoperative period will be necessary for HOCM patients who undergo noncardiac surgical procedures.

Funding Acknowledgement
Type of funding sources: None.
Influence of novel risk markers on defibrillator implantation in hypertrophic cardiomyopathy
Authors: Procter W T C , Husselbury T , Michailidou S , Dhinoja M B , Petersen S E , O'Mahony C , Mohiddin S A , Malcolmson J W 
Published: October 2022

Abstract

Background/Introduction
To guide implantable defibrillator (ICD) use in hypertrophic cardiomyopathy (HCM), European Society of Cardiology (ESC) guidelines recommend using individualised sudden death (SCD) risk scores based on quantitative clinical data. Newly published American guidelines are based on the accumulation of binary risk markers, which include imaging-based novel risk markers (NRMs) that were absent from the development of the ESC's algorithm. These NRMs are ejection fraction &lt;50%, apical aneurysm, and extensive late gadolinium enhancement on cardiac magnetic resonance (CMR) imaging.

Purpose
To assess how NRMs may have altered ICD prescription across ESC-based SCD risk status prior to publication of current American guidance.

Methods
We examined electronic records (2013–2020) of a subset of HCM patients with contemporaneous (within 12 months) CMR and echocardiography data for NRMs, ESC risk status, and ICD prescription. Differences in categorical data were assessed by Fisher's exact test.

Results
We studied 334 HCM patients (74% male; age: 58±14 years), of whom 83 (25%) were referred for ICD. ESC risk status was considered low, medium (4–6% 5-year SCD risk), or high in 264, 26, and 20 patients, for whom ICDs were recommended in 40 (15%), 20 (77%), and 18 (90%) patients, respectively. In patients with low SCD risk status, rate of ICD recommendation was significantly higher when ≥1 NRMs were present (34/126 – 27% vs. 0 NRMs: 6/138 – 4%; p&lt;0.0001). NRMs did not appear to influence ICD recommendation in patients with medium (≥1 NRMs: 14/17 – 82% vs. 0 NRMs: 6/9 – 67%; p=0.6) or high (≥1 NRMs: 14/15 – 93% vs. 0 NRMs: 4/5 – 80%; p=0.4) SCD risk status (Figure 1). NRMs were less frequent in low risk patients than in high risk patients (126/264 – 48% vs. 15/20 – 75%; p&lt;0.05), suggesting interaction between ESC status and NRMs (Figure 2).

Conclusion
NRMs have disproportionate influence on ICD prescription in low ESC risk HCM patients. However, NRMs are not independent of ESC risk status, suggesting iterative development of the ESC's algorithmic approach will be the most effective way of predicting SCD.

Funding Acknowledgement
Type of funding sources: None.
Association between obesity and hypertrophic cardiomyopathy phenotype, validation in a latin american cohort
Authors: Quiroga A , Zonis L , Veron L F , Casabe J H , Moukarzel J A , Banchio Dal Bo J , Fanilla M , Cutine P , Litwak N , Fernandez A 
Published: October 2022

Abstract

Objectives
Hypertrophic Cardiomyopathy (HCM) is the most common genetic cardiomyopathy. The influence of obesity on the disease phenotype is not well established. A recent publication sought to answer this gap. Our objective is to assess the relationship of a obesity on the phenotype of our cohort and compare it to the published literature.

Methods
We performed a retrospective observational study of consecutive patients diagnosed with HCM at a single center in Argentina. We included 1331 patients between March 1993 and December 2021. We excluded patients &lt;18 years of age, metabolic diseases & syndromes and patients who were lost on follow up. We defined Obesity as a body mass index ≥30 kg/m2.

Results
1168 patients had available data for analysis. 54 (10.4%) patients were obese. Mean age was 54±28 years and 33 (61%) were male. Average maximal left ventricular wall thickness (LVWT) in this group was 20 mm ± 5 mm. When comparing obese vs non-obese patients we found no statistically significant differences for gender, age, family history of HCM, family history of sudden death (SD), previous VF/VT/NSVT, ESC low, intermediate or high risk for SD groups, fibrosis, apical aneurism, LV diameter, maximum LVWT, high risk genetic mutation, abnormal BP response to exercise, device implant, septal alcohol ablation, reduced EF on follow up, nor death/HF/Stroke/AICD appropriate therapy/AF development. We did find that obese patients had less unexplained syncope (6% vs 13%, OR 0.43, p=0.03), had more frequently an NYHA &gt; III/IV (15.3% vs 8.9%, OR 1.8, p=0,03), a trend to having less frequently a LVWT &gt;30 mm (3.5% vs 8.1%, OR 0.41, p=0.07) but more frequently had systolic anterior motion of the mitral valve on echocardiography (45% vs 34%, OR 1.58, p=0.04). We noted a trend to being more frequently indicated LV myectomy (9.2% vs 5%, OR 1.9, p=0,07) and significantly having elevated ultrasensitive troponin I (7.8% vs 3.7%, OR 2.2, p=0,04).

Conclusions
We found that obese patients from our cohort, as described in other series, had more advanced symptoms. In our set of obese patients they were more frequently sent to myectomy rather than alcohol septal ablation as described in other series. AF was not more prevalent among our obese patients and maximum wall thickness and fibrosis were not higher either. We did find that obese patients had more frequently elevated biomarkers. As HCM is so frequent it is important to characterize cohorts and compare data.

Funding Acknowledgement
Type of funding sources: None.
Natural history and clinical parameters of chemotherapy-induced dilated cardiomyopathy
Authors: Lalario A , Del Mestre E , Lo Casto M , Nuzzi V , Manca P , Bromage D , Barbati G , Merlo M , Sinagra G , Cannata A 
Published: October 2022

Abstract

Background
Chemotherapy-induced dilated cardiomyopathy (CI-DCM) is a well-recognized phenotype of non-ischemic dilated cardiomyopathy (DCM), characterized by poor outcomes. However, a detailed comparison between idiopathic DCM (iDCM) and CI-DCM is still lacking.

Methods
All consecutive DCM patients enrolled in the Trieste Muscle Heart Disease Registry were analyzed. CI-DCM and iDCM were defined according to current recommendations. The primary study outcome measure was all-mortality death and secondary outcomes were a) a composite of cardiovascular death/heart-transplantation/ventricular-assist-device implantation, and b) major ventricular arrhythmias.

Results
The study included 551 patients (499 iDCM and 52 CI-DCM). At enrolment, compared to iDCM, CI-DCM patients were older (51±14 years vs 58±3 years respectively, p&lt;0.001) and had a higher left ventricular ejection fraction (35%±10 vs 32%±9, p=0.03). Over a median follow-up of 90 months (IQR 54–140 months), CI-DCM patients had a higher incidence of all-cause mortality compared to iDCM (36.5% vs 8.4% in CI-DCM and iDCM respectively, p&lt;0.001), while the incidence of major ventricular arrhythmias was higher in the iDCM group compared to CI-DCM (4% vs 0%, in CI-DCM and iDCM respectively, p=0.03). The risk of the composite outcome was comparable between the two groups (p=0.91). At Cox multivariable analysis, the diagnosis of CI-DCM emerged as independently associated to primary outcome (HR 6.42, 95% CI 2.52–16.31, p&lt;0.001).

Conclusions
In a well-selected DCM cohort, patients with a chemotherapy-induced aetiology had a higher incidence of all-cause mortality compared to iDCM. Conversely, the incidence of life-threatening ventricular arrhythmic events was higher among patients with iDCM.

Funding Acknowledgement
Type of funding sources: None.