bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical and electrocardiographic differences between apical and midventricular stress cardiomyopathy
Authors: Esteban-Lucia L , Martinez-Milla J , Franco-Pelaez J A , Lopez-Castillo M , Martin-Reyes R , Palfy J , Romero-Daza A , Pinero Lozano A , Avila Barahona P , Kallmeyer Mayor A M , Gonzalez Lorenzo O , Tunon J 
Published: October 2021

Abstract

Background
Stress cardiomyopathy (SCM) is a transient ventricular dysfunction frequently precipitated by acute emotional or physical stress. According to ventricular contractile pattern there are four SCM variants, being apical (80%) and midventricular (15%) the most frequents. Our aim is to describe the differences between these two SCM variants.

Methods
We performed an observational study of patients who were diagnosed with SCM in our centre from February 2010 to January 2020. We registered demographic information, medical history, clinical, echocardiographic and analytical data of our patients and we performed a digital analysis of the 12-lead electrocardiogram (ECG) recorded in the first 48 hours after the onset of symptoms. Patients were divided into two groups, those with apical LV ballooning and patients with midventricular SCM.

Results
We included 86 patients; baseline characteristics of the population are described in the table. The median age was 77 (IQR: 67.7–82.9), and 91.9% were female. Fifty-six patients (65.1%) had apical variant whereas thirty patients (34.9%) showed midventricular one. The most frequent antecedent trigger was an emotional stressor (36.3%) follow by physical stressor (identified in 23.8%). Chest pain was the most frequent clinical presentation (54.7%). Median LVEF was 35% (IQR 30.0–45.0) and mean basal septal thickness was 9.50 (IQR 9.0–11.0). Median peak of troponin level was 1.71 (IQR 0.6–3.4).
Comparing apical and midventricular variants of SCM median age was 80.2 (IQR 68.9–84.5) in apical group and 71.6 (IQR 59.64–78.0) in midventricular variant (P=0.001). There were no other differences in the medical history, clinical, echocardiographic and analytical data between both groups (table). After comparative analysis of ECG we found differences in negatives T wave location and QT duration. Apical variant present more negative-T waves in leads II, III, AVF, V4, V5, V6, while in midventricular variant they are more present in AVR and AVL (figure 1). Mean QT duration was 508.0ms (IQR 470.0–552.0) in apical variant and 470.5ms (IQR 423.7–524.2) in midventricular group (p=0.026). There were no differences in Q waves between both groups.

Conclusions
In our group of patients, apical and midventricular stress cardiomyopathy variants exhibit clinical and electrocardiographic differences. Patient with apical stress cardiomyopathy are older. They present more negative T waves in inferior and precordial leads, and longer QT interval. These results suggest that the pathophysiology of both entities could be different.

Funding Acknowledgement
Type of funding sources: None. Negative T-Waves comparationPopulation characteristics.
Right ventricular dysfunction before transcatheter aortic valve implantation: incidence, predictive factors and prognostic impact
Authors: Sacri C , Durand E , Tron C , Barbe T , Hemery T , Burdeau J , Dacher J N , Eltchaninoff H 
Published: October 2021

Abstract

Background
Right ventricular dysfunction (RVD) is considered to be a late marker of advanced aortic stenosis (AS) and is associated with poor prognosis. Currently. there are conflicting data on the impact of RVD on clinical outcomes in patients with severe AS treated with TAVI. Moreover, few studies have studied the evolution (recovery or persistence) of RVD and its prognostic impact.

Objectives
To assess the incidence and predictive factors of RVD before TAVI, its prognostic impact and its evolution after TAVI.

Methods
All patients treated with TAVI for severe AS were included in a prospective single center database. Only patients who had a quantitative assessment of RV including Tricuspid Annular Plane Systolic Excursion (TAPSE) and/or doppler tissue imaging-derived tricuspid lateral annular systolic velocity (S') measurements, were eligible to this study. RVD was defined by a TAPSE &lt;17 mm or S' &lt;9.5 cm/s if TAPSE was not available.

Results
Between May 2014 and April 2019, 503 patients with RV function evaluation were included. Incidence of RVD before TAVI was 18.7%. Predictors of RVD were diabetes (P=0.03), atrial fibrillation (P=0.001), altered left ventricular ejection fraction (P&lt;0.0001), left ventricular dilatation (P=0.007), and previous cardiac surgery (P=0.002). Long-term survival was altered in patients with RVD before TAVI as compared to those without RVD (HR 1.97, 95% CI: 1.1–3.4, P=0.01). One year after TAVI, 58.7% of patients with baseline RVD had a normal RV function and had similar outcome as compared to those without RVD at baseline. In contrast, patients with persistent RVD had the worst prognosis.

Conclusions
RVD is not rare and has a deleterious prognostic impact in patients treated by TAVI. Recovery of normal RV function is frequent after TAVI whereas persistence of RVD is associated with poor outcomes.

Funding Acknowledgement
Type of funding sources: None.
TAVR in patients with low-flow low-gradient aortic stenosis – outcome data after three years from one large centre
Authors: Steffen J , Reissig N , Zadrozny M , Fischer J , Andreae D , Braun D , Orban M , Theiss H , Peterss S , Hausleiter J , Massberg S , Deseive S 
Published: October 2021

Abstract

Background
The outcome of patients with low-flow low-gradient (LFLG) aortic stenosis after transcatheter aortic valve replacement (TAVR) is not well evaluated. Long-term clinical success is thought to be less pronounced in LFLG patients compared to patients with high gradient (HG) aortic stenosis.

Purpose
The purpose of this study was to characterise different LFLG groups and determine their outcome after TAVR. We hypothesised that there would be relevant differences in baseline characteristics and patient survival after TAVR.

Methods
All patients undergoing TAVR for severe aortic stenosis at our centre between 2013 and 2019 were included in the study. Patients have been split into groups according preinterventional echocardiography data according to mean pressure gradient (dPmean), ejection fraction (EF), and stroke volume index (SVi). Patients with a dPmean &lt;40 mmHg and SVi ≤35 ml/m2 were subdivided into classical low-flow low-gradient (cLFLG, EF &lt;50%) and paradoxical low-flow (pLFLG, EF ≥50%). Patients with previous aortic valve replacement or severe aortic regurgitation were excluded from the analysis.

Results
1,772 patients were analysed (mean follow-up 2.2 years, median age 81.7 [77.5–85.7] years) and split into groups: HG, 953 patients (54.3%), cLFLG, 446 patients (25.2%), and pLFLG 373 patients (21.1%). Baseline characteristics showed significant differences (p&lt;0.01), among others, in sex (male sex, HG 46.1% vs. cLFLG 69.5% vs. pLFLG 44.5%), rate of atrial fibrillation (HG 20.3% vs. cLFLG 36.3% vs. pLFLG 41.6%), coronary artery disease (HG 56.2% vs. cLFLG 73.5% vs. pLFLG 63.4%), and grade 3 or 4 mitral regurgitation (HG 2.2% vs. cLFLG 5.5% vs. pLFLG 6.8%). Accordingly, Society of Thoracic Surgeons (STS) Scores differed significantly: HG, 3.0 [2.0–5.0], cLFLG, 5.0 [3.0–7.3] pLFLG, 3.9 [2.2–6.0] (p&lt;0.01).
Rates of periprocedural complications including death, device failure, pericardial effusion, stroke or myocardial infarction were comparable between groups. Mortality rate (figure 1) was highest for cLFLG patients (43.4% [95% confidence interval, 37.3–48.6%]) compared to HG (25.1% [21.6–28.5%]) or pLFLG (32.9% [26.9–38.4%]), Log-rank test, &lt;0.001. Corresponding hazard ratios were 2.1 [1.7–2.6] (p&lt;0.001) for cLFLG and 1.5 [1.2–2.0] (p&lt;0.001) for pLFLG. Similar results were obtained when adjusting to STS score (figure 2).

Conclusion
In this all-comer analysis, almost half of the patients belong toLFLG groups with considerable differences in patient characteristics. While equally safe during the procedure, patients with LFLG aortic stenosis show increased 3-year mortality rates compared to patients with HG aortic stenosis. Further studies evaluating this are needed.

Funding Acknowledgement
Type of funding sources: None. Figure 1. 3-year mortalityFigure 2. STS score-adjusted mortality
Invasive and Doppler Transvalvular gradients after transcatheter aortic valve replacement
Authors: Fugar S , Deka K , Anderson C , Lama Von Buchwald C , Geroux R , Al-Amoodi M , White J , Kavinsky C 
Published: October 2021

Abstract

Background
Differences in mean gradients after Transcatheter valve replacement (TAVR) vary depending on the valve type and the modality used to measure the gradients. Currently there is a paucity of data on the relationship between invasive and doppler derived gradients after TAVR.

Purpose
We sort to assess the difference in doppler, and catheter derived aortic valve gradients after TAVR

Methods
This is a single center retrospective study using consecutive patients who presented for TAVR on account of native aortic valve stenosis at our institution from May 2012 till December 2020. Patients with both intraoperative invasive and postoperative doppler derived pressure gradients were included in the analysis. Student T-test were used to compare mean gradients. Pearson's correlation test was used to examine the correlation between measured gradients.

Results
A total of 587 patients were included in our study. Fifty one percent were male and 462 (78.7%) underwent TAVR with a balloon expandable valve. In the entire cohort the mean gradient measured invasively was significantly lower than those measured by echo doppler (4.48±3.25 vs. 5.57±3.11, P&lt;0.001). There, however, was a positive correlation between invasive and doppler measured gradients (figure 1). In those who received balloon expandable valves, the invasive gradient was 4.39±3.30 and the doppler derived gradient was 5.47±3.04 (P&lt;0.001), while in those self-expanding valves, the invasive gradient was 4.81±3.04 and doppler derived gradient was 5.94±3.36 (P&lt;0.001).

Conclusion
Post TAVR gradients were all significantly lower when measured invasively as compared to those measured using doppler. Self-expanding valves overall had higher residual gradients. Further studies are needed to assess the correlations between invasively measured gradients and clinical outcomes post TAVR.

Funding Acknowledgement
Type of funding sources: None.
Left ventricular hypertrophy secondary to Fabry disease: mechanical dispersion and arrhythmic burden
Authors: Menezes Fernandes R , Azevedo O , Cordeiro F , Lourenco A 
Published: October 2021

Abstract

Introduction
Fabry disease (FD) is an X-linked progressive and multisystemic disease, that could lead to left ventricular hypertrophy (LVH). Mechanical dispersion (MD) has been associated to risk of ventricular dysrhythmias and sudden death in several cardiomyopathies.

Purpose
To evaluate MD and arrhythmic burden in FD patients with and without LVH.

Methods
We conducted a prospective study encompassing FD patients followed in a Reference Center of Lysosomal Storage Disorders. All patients performed a complete echocardiographic evaluation, including left ventricular (LV) strain analysis by two-dimensional speckle tracking imaging. Clinical characteristics, echocardiographic parameters and 24h-holters results were analysed. FD patients were divided in two groups according to the presence or absence of LVH. Both groups were compared by Chi-square test for categorical variables and Student's T-test for continuous variables. The significance level was 0,05.

Results
A total of 91 FD patients were included, with 62,6% of female predominance and 36,3% of patients with LVH. Patients with LVH were older (63,6 vs 44,2 years-old; p&lt;0,001), were predominantly male (60,6% vs 24,1%; p=0,001), had a larger prevalence of arterial hypertension (63,6% vs 10,3%; p&lt;0,001), coronary heart disease and stroke (both 9,1% vs 0%; p=0,02). In FD patients with LVH, mean interventricular septum thickness was 16,4 mm, mean LV mass index was 157,7 g/m2, mean left atrial volume index was 34,4 ml/m2 and mean E/e' was 12,6, with 63,6% of these patients having diastolic dysfunction. Global longitudinal strain (GLS) was lower in patients with LVH (−15,6% vs −19,6%; p&lt;0,001), with a higher base-to-apex longitudinal strain gradient (9,3% vs 6,5%; p=0,013). Global radial and circumferential strains were not statistically different between both groups. Interestingly, twist was higher in patients with LVH (17,5° vs 13,8°), despite the lower GLS, but it did not reach statistical significance (p=0,077). MD was higher in patients with LVH (72,4 vs 38,3 ms; p&lt;0,001), as well as the prevalence of premature ventricular contractions (PVC) (93,8% vs 61,8%; p=0,001). Non-sustained ventricular tachycardia (NSVT) was also more prevalent in patients with LVH (18,8% vs 9,1%), although not reaching statistical significance (p=0,191).

Conclusion
FD patients with LVH present lower GLS, with a higher base-to-apex longitudinal strain gradient and higher MD, compared to FD patients without LVH. Although PVC were significantly more frequent in FD patients with LVH, NSVT were not statistically different between both groups.

Funding Acknowledgement
Type of funding sources: None.
Mortality in patients with isolated congenital complete atrioventricular block in Sweden: a register-based, nationwide cohort study
Authors: Shao Y , Kok W , Eriksson P , Johansson B , Dellborg M , Mandalenakis Z 
Published: October 2021

Abstract

Purpose
The congenital heart block is a rare but potentially serious heart conduction disease and associated with high mortality during the fetal or neonatal periods. Earlier reports are based on relatively small number of patients and there are limited data about long-term outcomes.

Methods
Data were collected from the Swedish Patient and Cause of Death register to identify all patients who were born and diagnosed with isolated congenital complete atrioventricular block (CCAVB) between 1970 and 2017. Each patient with isolated CCAVB was matched with 10 control individuals, by birth year and sex from the Total Population Register.

Results
Totally 506 patients with CCAVB and 5,050 controls included in the study (55.3% males). The incidence rate of mortality in patients with isolated CCAVB was 166 per 10.000 person-years. The overall risk of mortality in patients with isolated CCAVB was 45.4 times (95% confidence interval (CI) 32.7–62.9) higher compared to matched controls. In a subgroup investigation, the highest risk of mortality was found in patients with isolated CCAVB from birth until the age of 17 (hazard ratio 80.8, 95% CI 51.6–126.4), compared to matched controls without CCAVB.

Conclusion
In this nationwide cohort study, patients with isolated CCAVB was associated with more than 40-fold risk for overall mortality compared to age and sex matched controls. The greatest risk was found during childhood. Regularly follow-up in tertiary centers and the aware of high risk of complications may be the key to prevention.

Funding Acknowledgement
Type of funding sources: Public grant(s) – National budget only. Main funding source(s): The Swedish Government, the Swedish Research Council
Anomalous origin of the right coronary artery with interarterial course: red flag or innocent bystander?
Authors: Albuquerque F , De Araujo Goncalves P , Ferreira A , Lopes P , Dores H , Marques H , Freitas P , Goncalves M , Cardim N 
Published: October 2021

Abstract

Aims
Anomalous origin of the right coronary artery from the opposite sinus (right-ACAOS) with interarterial course (IAC) has been associated with increased risk of sudden cardiac death (SCD). Widespread use of coronary computed tomography angiography (CCTA) has led to increasing recognition of this condition, even among healthy individuals. Our study sought to examine the prevalence, anatomical characteristics and outcomes of right-ACAOS with IAC in patients undergoing CCTA for suspected coronary artery disease (CAD).

Methods and results
We conducted a retrospective analysis of consecutive patients referred for CCTA at one tertiary hospital from January 2012 to December 2020. Right-ACAOS with IAC patients were analyzed for cardiac symptoms and long-term occurrence of first MACE (SCD, non-fatal myocardial infarction (MI) or revascularization of the anomalous vessel). CCTAs were reviewed for anatomical high-risk features and concomitant CAD. Among 10928 patients referred for CCTA, 28 patients with right-ACAOS with IAC were identified. Mean age was 55±17 years, 64% were male and 11 (39.3%) presented with stable cardiac symptoms. Most patients had at least one high risk anatomical feature. During follow-up, there were no CV deaths or aborted SCD episodes and only 1 patient underwent surgical revascularization of the anomalous vessel.

Conclusion
Right-ACAOS with IAC is an uncommon finding (prevalence of 0.26%). In a contemporary population of predominantly asymptomatic patients who survived this condition well into adulthood, most patients were managed conservatively with a low event rate. Additional studies are needed to support medical follow-up as the preferred option in this setting.

Funding Acknowledgement
Type of funding sources: None.
Screening for Fontan-associated liver disease: are we doing enough?
Authors: Tsoi V , Mitropoulou P , Chatterjee K , Lwin M , Salmon A , Smith L , Fitzsimmons S , Kyi N , Carroll A 
Published: October 2021

Abstract

Introduction
Fontan-associated Liver Disease (FALD) is an extracardiac complication with a substantial disease burden that can reduce quality of life, increase healthcare demand and result in premature mortality. Effective FALD screening is paramount in preventing late-stage complications.

Purpose
To assess the adherence of a tertiary adult congenital heart disease referral centre to FALD screening.

Methods
1. A total of 256 Fontan patients reviewed at our centre between 1st January 1977 and 31st December 2020 were identified from the congenital cardiac surgical database and the adult congenital heart disease clinical nurse specialist database.
2. The following patients were excluded: aged under 18 years on 31st December 2020 (103 cases), out of region or loss to follow-up (35 cases), death (30 cases), cardiac or liver transplantation (4 cases) and Fontan takedown (3 cases.) The mean endpoint (death, transplant or Fontan takedown) was 30 years.
3. The final study population included 81 patients.
4. Data was collected by review of clinic letters, blood test and imaging results from electronic records between 1st January 2018 and 31st December 2020.
5. The data were analysed to determine whether the screening recommendations from “Fontan-Associated Liver Disease: Proceedings from the American College of Cardiology Stakeholders Meeting, October 1 to 2, 2015, Washington DC” (ACC Stakeholders Meeting) were performed.

Results
The ACC Stakeholders Meeting recommended history taking and physical examination at least once a year with Fontan blood tests (full blood count, urea and electrolytes, liver function tests, alpha-fetoprotein and international normalized ratio) and imaging (abdominal ultrasound, computerised tomography or magnetic resonance imaging) at least once every three years.
Of the 81 patients (47 male, mean age 27±8.3 years), the mean age of Fontan completion was 6.6±5.3 years. The mean follow-up period since Fontan completion was 20±8.1 years. History taking and physical examination were documented in 84% (68/81) and 79% (64/81) of patients respectively. 60% (59/81) had a full set of Fontan blood tests taken which increased to 73% (49/81) if alpha-fetoprotein was excluded while imaging was performed in 80% (65/81) of cases.
Overall, 46% (37/81) cases had abnormal Fontan blood test or imaging. Hepatology referral was made in only 21% (17/81) of cases and MELD-XI score was not documented.

Conclusions
FALD screening tests were frequently underperformed compared to international guidance. Adherence may be improved by 1) promoting a Fontan blood test bundle; 2) implementing a standardised letter template which includes an investigation date list; 3) advocating MELD-XI score; and 4) exploring a joint cardiac and hepatology Fontan clinic.

Funding Acknowledgement
Type of funding sources: None. Figure 1Figure 2
Effects of varying shear stress profiles on the regulation of pulmonary artery endothelial cell gene expression: a focus on selected mediators of vascular tone, inflammation and angiogenesis
Authors: Mahomed A S , Burke-Gaffney A , Toe Q , Naser J , Quinlan G J , Wort S J 
Published: October 2021

Abstract

Background
Pulmonary arterial hypertension (PAH) is a complex pathology characterized by obliterative vascular remodeling that leads to right heart failure and death. Predisposition to PAH is associated with mutations in the BMPR2 gene in approximately 70–80% of familial cases and around 30% for that of sporadic PAH. The study of the pathogenetic basis of PAH is often performed in static endothelial cultures. Such two-dimensional, isolated cell microenvironments fail to consider the heterogeneity in mechanical stress acting on endothelial cells in various regions of the pulmonary vascular tree. In the remodeled pulmonary vasculature, low and oscillatory shear stress is observed in the proximal pulmonary artery with high shear stress in distal pre-capillary pulmonary arterioles. Therefore, the impact of varied shear profiles (including both laminar and oscillatory flow) on pulmonary artery endothelial cell (and that of BMPR2-deplete) gene expression of common vasoactive (EDN1, ENOS), proinflammatory (IL6, IL8) and angiogenic mediators (ANG2, VEGFA), are poorly described.

Purpose
To evaluate the effects of shear stress magnitude, including unidirectional and oscillatory flow on BMPR2-knockdown human pulmonary artery endothelial cell (HPAEC) gene expression of EDN1, ENOS, IL6, IL8, ANG2 and VEGFA.

Methods
HPAECs were transfected with siRNA directed against BMPR2 (siB2) or with a non-targeting control (siCon). Cells were exposed to 10 hours of laminar or oscillatory flow (1Hz; 1.5 dyn/cm2, 15 dyn/cm2 or 90 dyn/cm2) using a parallel-plate fluid flow chamber system. Measurement of mRNA expression was performed using qPCR.

Results
Shear stress intensity and flow type (unidirectional and oscillatory) mediated diverse effects on HPAEC gene expression across the markers studied. Changes in gene expression were calculated relative to that of static siCon-transfected HPAECs and in such a manner are summarized as fold changes in the table below. Asterisks are shown where significant fold differences are reported. *P≤0.05, **P≤0.01, ***P≤0.001, ****P≤0.0001. aP≤0.05, bP≤0.05, cP≤0.05, denote comparisons between groups. Of note, no significant differences in gene expression were observed between static siCon and static siB2.

Conclusions
For the markers studied, different magnitudes of shear stress and flow profiles (together with BMPR2 loss) exhibit varied patterns of gene expression in the pulmonary vascular endothelium. As such, this illustrates the need for wider study of in vitro endothelial-shear stress interactions in understanding mechanisms of remodeling in PAH.

Funding Acknowledgement
Type of funding sources: None. Table 1