bluuebunny/crossref_metadata_2025_split huggingface dataset:
Effects of early initiation of comprehensive heart failure therapy on echocardiographic parameters of young patients with newly diagnosed idiopathic dilated cardiomyopathy
Authors: Hassan KM , Chipamaunga T , Joubert LH , Doubell AF 
Published: February 2022

Abstract

Funding Acknowledgements
Type of funding sources: None.

Introduction
Heart failure with reduced ejection fraction (HFrEF) is a growing problem worldwide and is associated with significant morbidity and mortality. Angiotensin receptor neprilysin inhibitors (ARNI) and sodium-glucose cotransporter 2 inhibitors (SGLT2-i) are now seen as disease modifying agents with growing evidence to support their early initiation in order to prolong lives of patients with HFrEF. However, most of these trials were conducted in the developed world involving older patients with ischaemic cardiomyopathies, in contrast to younger patients with idiopathic dilated cardiomyopathies (DCMO) typically seen in Africa. Further, these drugs are expensive and remain out of reach for most patients in the developing world.

Purpose
To determine the effects of early initiation of comprehensive heart failure (HF) therapy consisting of ARNI, SGLT2-i, β-blocker and mineralocorticoid receptor antagonist on transthoracic echocardiography (TTE) parameters of patients with newly diagnosed idiopathic DCMO.

Methods
This is prospective cohort study. All patients presenting to a single tertiary centre in South Africa between January and July 2021 with newly diagnosed idiopathic DCMO that were initiated on a regimen of comprehensive heart failure therapy as first line therapy were included. TTE were performed at initiation and at monthly follow up visits.

Results
Eight patients (mean age 35.4 ± 7 years, 25% females) were included. At baseline, the mean left ventricular ejection fraction (LVEF) was 17.1 ± 3.2%, mean left ventricular end diastolic diameter (LVEDD) was 68.0 ± 7.6mm, mean E’ (average) 4.0 ± 1.9m/s, mean LV global longitudinal strain (GLS) -4.5 ± 1.9%, mean LVOT VTI 10.9 ± 3.2cm and mean TAPSE 14.3 ± 4.1mm. The patients were followed up for an average of 4 months (range 1 – 5 months). TTE performed at the latest follow up visit showed a significant increase in LVEF (21.3%; 95% CI 13.5 - 29.0; p = 0.01), average E’ (2.5m/s; 95% 0.3 - 4.7; p = 0.05), GLS (-6.7%; 95% CI -1.0 - 8.9; p = 0.01), LVOT VTI (5.0cm; 95% CI 1.4 - 8.5; p = 0.03) and TAPSE (7mm; 95% CI 2.6 - 11.4; p = 0.02), and a significant reduction in LVEDD (6.5mm; 95% CI 1.1 - 11.9; p = 0.03).

Conclusion
Early initiation of comprehensive HF therapy including ARNI and SGLT2-i is associated with significant improvements in left and right ventricular function and LV reverse remodelling in a small cohort of young South African patients with newly diagnosed idiopathic DCMO. This supports the widespread adoption of this therapeutic strategy, even in a resource limited setting, in a population of patients with limited access to other therapeutic options such as mechanical circulatory support and cardiac transplantation. Abstract Table 1 Abstract Figure 1
Left atrial myopathy is associated with exercise capacity and ventilatory efficiency in hypertrophic cardiomyopathy
Authors: Zegkos T , Kamperidis V , Gossios T , Ntelios D , Parcharidou D , Papanastasiou CA , Katranas S , Rouskas P , Karvounis H , Efthimiadis GK 
Published: February 2022

Abstract

Funding Acknowledgements
Type of funding sources: None.

Background
Data about the predictive value of left atrial (LA) myopathy for exercise capacity and ventilatory efficiency in patients with hypertrophic cardiomyopathy (HCM) are limited.

Purpose
The aim of this study was to evaluate the association of LA myopathy, evaluated by echocardiography LA strain, with exercise capacity and ventilatory efficiency, evaluated by cardiopulmonary exercise testing (CPET), in HCM patients.

Methods
In total, 241 consecutive HCM patients (51 ± 16 years old; 67.2% male) on sinus rhythm that underwent CPET and transthoracic echocardiography at the same visit were studied. Exercise capacity (maximal oxygen consumption/predicted [%peakVO2]) and ventilatory efficiency (ventilation/carbon dioxide output [VE/VCO2] slope) were assessed by CPET. LA myopathy was examined by speckle tracking myocardial deformation parameters: LA reservoir, conduit and booster strain.

Results
All the three LA strain values were univariate predictors of exercise capacity and ventilatory efficiency. Among them, LA reservoir strain had the higher r correlation coefficient for predicting both %peakVO2 and VE/VCO2 slope and was included in multivariable analyses. LA reservoir strain, male gender and E/E’ ratio were independent predictors of exercise capacity. LA reservoir strain, male gender, non-sustained ventricular tachycardia and LA volume index were independent predictors of ventilatory efficiency (Table).

Conclusion
LA myopathy, as reflected by the LA strain values, was associated with exercise capacity and ventilatory efficiency in HCM individuals. LA reservoir strain was the only common independent predictor for %peakVO2 and VE/VCO2 slope. Abstract Table
Left atrioventricular coupling index in hypertrophic cardiomyopathy and risk of new-onset atrial fibrillation
Authors: Meucci MC , Fortuni F , Galloo X , Bootsma M , Crea F , Bax JJ , Ajmone Marsan N , Delgado V 
Published: February 2022

Abstract

Funding Acknowledgements
Type of funding sources: None.

Background
In patients with hypertrophic cardiomyopathy (HCM) accurate risk stratification for new onset atrial fibrillation (AF) has important prognostic implications. Left atrioventricular coupling index (LACI) has been recently associated with the occurrence of AF in patients without history of cardiovascular disease.

Purpose
The objective of this study was to investigate the association between LACI and new onset AF in HCM patients and its incremental value over conventional left atrial (LA) parameters.

Methods
A total of 373 HCM patients without history of AF (48 ± 17 years, 66% men) were evaluated by transthoracic echocardiography. LACI was defined by the ratio of the LA end-diastolic volume divided by the LV end-diastolic volume. The cut-off value for LACI (≥40%) to identify LA-left ventricular (LV) uncoupling was chosen based on the risk excess of new-onset AF described with a spline curve analysis. Cox proportional hazard models were used to evaluate the association between LACI and the occurrence of AF.

Results
The median LACI was 38% (interquartile range: 24-56) and LA-LV uncoupling (LACI ≥40%) was observed in 171 (45.8%) patients. During a mean follow-up of 11.0 ± 5.6 years, 118 subjects (31.6%) developed new-onset AF. The cumulative event-free survival at 10 years was 53% for patients with LA-LV uncoupling (LACI ≥40%) versus 94% for patients without LA-LV uncoupling (LACI &lt;40%) (p &lt; 0.0001; Figure 1). Multivariable analysis showed an independent association between new-onset AF and LA maximum volume indexed (LAVImax) (hazard ratio [HR], 1.03; 95% CI, 1.02–1.04), LA minimum volume indexed (LAVImin) (HR, 1.04; 95% CI, 1.03–1.05), LA emptying fraction (HR, 0.97; 95% CI, 0.96–0.98) and LACI (HR, 1.02; 95% CI, 1.01–1.02; all p &lt; 0.0001). The inclusion of LACI in the multivariate model provided larger improvement in the risk stratification for new-onset AF, as compared to conventional LA parameters (Figure 2). Furthermore, the likelihood ratio test demonstrated incremental value of LACI assessment on the top of the multivariate model including LAVImin to predict new-onset AF (p = 0.02), while the addition of LAVImin did not improve the risk discrimination of the multivariate model including LACI (p = 0.36).

Conclusion
Greater LACI, indicative of LA-LV uncoupling, was independently associated with the occurrence of new-onset AF in patients with HCM and demonstrated a stronger risk discrimination power compared to conventional LA parameters. This simple ratio may be easily implemented in clinical practice to improve risk stratification for new-onset AF in HCM. Abstract Figure. Incident AF according to LACI Abstract Figure.
The presence and extent of coronary microvascular dysfunction is associated to the severity of cardiomyopathy in patients with Fabry disease
Authors: Graziani F , Lillo R , Leccisotti L , Bruno I , Ingrasciotta G , Marano R , Rovere G , Manna R , Pieroni M , Camporeale A , Lanza GA , Crea F 
Published: February 2022

Abstract

Funding Acknowledgements
Type of funding sources: None.

Background
Coronary microvascular dysfunction (CMD) occurs before left ventricular hypertrophy (LVH) in Anderson Fabry Disease (AFD). Few data exist about the role of CMD in Fabry cardiomyopathy, when overt LVH has already established.

Purpose
Aim of our study was to assess the relationship between CMD and clinical and echocardiographic features in a cohort of Fabry cardiomyopathy patients.

Methods
We performed coronary CT scan to exclude epicardial coronary artery disease (CAD) in 27 AFD cardiomyopathy patients with angina and/or evidence of silent ischemia at treadmill stress test. All consenting patients with no CAD (n = 17) were submitted to resting and stress 13N-Ammonia myocardial perfusion PET/CT to assess the presence of CMD. All patients also underwent complete echocardiography. Patients were followed-up for 17.3 ± 12.5 months.

Results
Global coronary flow reserve (CFR) resulted &lt;2.5 in 7 (41%) patients. Global stress myocardial blood flow (MBF) was &lt;1.85 mL/min/g in 5 (29%) patients. Global transmural perfusion gradient (TPG, subendocardial MBF/subepicardial MBF) during stress was &lt;1.0 in 13/17 (76.5%) patients. Resting global TPG was ≥1 in 16 (94%) patients. Patients with CFR &lt; 2.5 were older (p = 0.02), had more severe LVH (maximal wall thickness p = 0.04), worst global longitudinal strain (p = 0.03) and E/e’ (p = 0.04) and higher troponin levels (p = 0.002) as compared to patients with CFR ≥ 2.5. They also performed less at treadmill stress (METs p = 0.045). No variables were associated to major cardiovascular events at multivariable analysis.

Conclusions
In Fabry cardiomyopathy patients with angina and/or evidence of silent ischemia, the prevalence of CMD is high and it is associated to a more severe cardiac phenotype, including cardiac biomarker and functional capacity. We are not able to draw any conclusion on the possible prognostic role of CMD in Fabry cardiomyopathy.
Myocardial work in hypertrophic cardiomyopathy - a new non-invasive parameter for segmental myocardial function?
Authors: Batzner A , Seggewiss H , Hahn P , Sahiti F , Maack C , Gerull B , Stoerk S , Morbach C 
Published: February 2022

Abstract

Funding Acknowledgements
Type of funding sources: Other. Main funding source(s): DFG

Introduction
Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiac disease. Alcohol septal ablation (PTSMA) is an established treatment option for patients with intracavitary obstruction. In non-obstructive patients, measurement of myocardial work (MW) using pressure strain loops was introduced as a new non-invasive tool to estimate myocardial performance.

Purpose
In this preliminary study, we compared global constructive work (GCW) and global wasted work (GWW) as well as the respective values for the septal- and lateral-basal segments in HOCM after successful PTSMA and non-obstructive HCM.

Methods
We included 12 patients (4 women, mean age 59.1 ± 17.6 years) with non-obstructive (maximal left ventricular gradient &lt;30 mmHg; mean 10.6 ± 6.2 mmHg) HCM. 7 (58%) patients (subgroup A) had successful gradient reduction by PTSMA ≥3 months before analysis, whereas 5 patients had non-obstructive HCM (subgroup B). Using echocardiographic measurements of longitudinal strain and non-invasive measurements of peripheral- and calculated (PWA) central blood pressure (BP) as left ventricular systolic pressure (LVSP), we estimated and compared GCW and GWW as well as constructive work (CW) and wasted work (WW) of septal-basal and lateral-basal segments.

Results
In subgroup B, NTproBNP was significant higher than in subgroup A (2050 ± 1957 vs. 552 ± 845 pg/ml; p &lt; 0.05). There were no differences between both subgroups in maximal septal thickness (20.7 ± 2.9 mm (A) vs. 22.2 ± 3.6 mm) and left atrial volume (91.6 ± 33.0 (A) vs. 114.0 ± 49.8 ml (B)). Using the measured peripheral BP as LVSP, GCW (1653 ± 347 (A) mmHg% vs 1641 ± 698mmHg%) and GWW (171 ± 89mmHg% (A) vs. 200 ± 126 mmHg%) showed no differences between both subgroups. Compared to published data of a healthy population, values of GCW were lower and values of GWW higher in HCM. Comparison in segmental analysis in subgroup A showed lower CW in septal-basal than in lateral-basal segments (1032 ± 385mmHg% vs. 1929 ± 699 mmHg%). In the subgroup B the finding was the same pattern (1024 ± 504mmHg% vs. 2301 ± 1069 mmHg%). In contrast, WW showed no difference between basal-septal and lateral-basal segments in both subgroups.

Conclusions
Our preliminary data show decreased GCW and increased GWW in HCM patients without obstruction at rest. As expected, we found lower CW in septal-basal segments than lateral-basal in patients after PTSMA with basal-septal-induced therapeutic infarction. Surprisingly, we found the same pattern in non-obstructive HCM patients. This finding might be one pathophysiological reason for not developing LVOT obstruction, which should be investigated in further detail.
Clinical outcomes of adults with Ebstein anomaly: 20 years of a single-center experience
Authors: Nartowicz S , Cieplucha A , Wasniewski M , Bartczak-Rutkowska A , Miechowicz I , Trojnarska O 
Published: February 2022

Abstract

Funding Acknowledgements
Type of funding sources: None.

Background
Ebstein anomaly (EA) is rare congenital heart disease. Malformation of the tricuspid valve and right ventricle may contribute to supraventricular arrhythmias, heart failure, and pacemaker implantation, affecting these patients" overall survival.

Aim
We aimed to evaluated long-term outcomes in adults with EA.
Methods and results: We retrospectively analyzed EA patients followed up in our center from 1988 to 2020. Seventy-one adults with EA were identified (40 [56.3%) males), including 11 (15.5%) patients operated on during childhood. The median age was 53 (range 24-84) years, and the median follow-up time was 7 (range 1-28) years. During this time, 40 (56.3%) individuals presented with supraventricular tachycardia, of whom 18 (45.0%) pts required at least one catheter ablation. Out of 18 patients treated invasively, only 7 (38.9%) patients were free from SVT after the first ablation; 7 pts (38.9%) underwent 2 ablations; 6 pts (33.3%) - required 3 and 4 ablations. Atrio-ventricular block of II or III degree was diagnosed in 11 (15.5%) adults. A total of fifteen (21.1%) individuals had a pacemaker implanted – 10 due to the AV block and 5 due to the sinus node dysfunction. The functional NYHA class was ≥ only in 15 (21.1.%) pts. In contrast, a cardiopulmonary test performed in majority of the study group (45 [63.4%] pts) revealed a reduced peak oxygen uptake (peakO2 &lt; 85% of predicted value) in 38 (84.4%), with a significant reduction (%peakO2 &lt; 60%) present in 13 (28.9%) patients. Ten patients (14.0%) died - 5 deaths were due to heart failure, 2 - sudden cardiac death, and thromboembolism, respectively, 1- due to myocardial infarction. The overall survival was 95% to age 50 years, 82% to age 60 years, 72% to age 70 years, and 53% to age 80 years.

Conclusion
Patients with EA are at high risk of developing supraventricular arrhythmias, heart failure and conduction disturbances requiring pacemaker implantation. However, a long-term prognosis of adults with EA is satisfactory.
Advanced echocardiographic function analysis in adolescents and young adults with corrected and residual coartation of the aorta
Authors: Lemos M , Rato J , Mata M , Sequeira M , Cordeiro Mendes S , Carmo Mendes I , Anjos R 
Published: February 2022

Abstract

Funding Acknowledgements
Type of funding sources: None.

Introduction
This study aimed to assess systolic and diastolic heart function changes in patients with history of aortic coarctation using advanced echocardiographic imaging. Additionally, we sought to analyse which severity factors influenced these changes.

Methods
We performed a complete echocardiographic evaluation, with advanced functional analysis, including myocardial work analysis, to a random sample of 53 patients (age 12 to 40 years). These had a previous history of coarctation of the aorta (CoAo), which was either corrected (aortic transisthmic Doppler gradient (Dgrad) ≤20mmHg) or presented a significant residual gradient (Dgrad &gt;20mmHg). A control group of healthy individuals, matched for age, sex and BMI, was subjected to the same evaluation. Selected dependent variables were: E/A, E’, E/E’, atrial strain parameters, biplane ejection fraction, ventricular global longitudinal strain, and global myocardial work (GMW). One-way ANOVA with appropriate post-hoc tests was done to compare the distribution of dependent variables among controls (n = 31), patients with corrected coartation (cCOAO) (n = 36), and patients with residual coartation (rCOAO) (n = 17). Multivariable linear regression was used to evaluate the association, in the 53 patients, between the dependent variables and parameters of CoAo severity: systolic blood pressure (SBP), left ventricular indexed mass (LVmass), Dgrad, and the ratio of the narrowest diameter of the aortic arch to the aorta at the diaphragm level (Aoratio). Statistical significance was established as p &lt; 0.05.

Results
Patients with either cCOAO or rCOAO had lower E’ (p &lt; 0.001), higher E/E’ (p &lt; 0.001), lower atrial reservoir (p &lt; 0.001) and conduit (p &lt; 0.001) strain, when compared with controls (table 1). Patients with rCOAO had higher GMW when compared with either cCOAO or controls (p = 0.002). Multivariable regression analysis showed that both lower atrial reservoir and conduit strain were associated with a narrower aortic arch (lower Aoratio (p = 0.002 and p = 0.011, respectively); higher E/E’ with higher LVmass (p = 0.030); higher GMW with higher LVmass (p = 0.027) and Dgrad (p = 0.035). Patients subsequently submitted to an intervention for coartation treatment (n = 8) had lower atrial conduit (p = 0.007) and higher GMW (p = 0.015) when compared to all other patients (n = 45). Conclusion: Myocardial work emerged as a particularly useful tool as it was both significantly different between CoAo groups, and significantly higher in more severe patients, driven by the LV mass and residual gradient. This analysis may have a role in these patients’ clinical decision-making. Abstract Table 1
Functional echocardiographic and serum biomarker changes following surgical and percutaneous atrial septal defect closure in children
Authors: Van Der Ven J , Van Den Bosch E , Kamphuis VP , Terol C , Gnanam D , Bogers AJJC , Breur JMPJ , Berger R , Blom NA , Koopman L , Ten Harkel ADJ , Helbing WA 
Published: February 2022

Abstract

Funding Acknowledgements
Type of funding sources: Foundation. Main funding source(s): Dutch Heart Foundation

Background
Ventricular performance is temporarily reduced following surgical atrial septal defect (ASD) closure. Cardiopulmonary bypass and changes in loading conditions are considered important factors, but this phenomenon is incompletely understood. We aim to characterize biventricular performance following surgical and percutaneous ASD closure and to relate biomarkers to ventricular performance following intervention.

Methods
In this multicenter prospective study, children scheduled for surgical or percutaneous ASD closure were included. Subjects were assessed pre-operatively (T1); in the 2nd week post-intervention (T2) and 1 year post-intervention (T3). At each time point an echocardiographic study and a panel of biomarkers was obtained.

Results
Sixty-three patients (age 4.1 [3.1–6.1] years) were included. Forty-three patients underwent surgery. At T2 right ventricular (RV) global longitudinal strain (GLS) was decreased for the surgical, but not the percutaneous, group (-17.6 ± 4.1 versus -27.1 ± 3.4, p&lt;.001). A smaller decrease was noted for left ventricular (LV) GLS at T2 for the surgical group (surgical -18.6 ± 3.2 versus percutaneous -20.2 ± 2.4, p=.040). At T3 LV performance returned to baseline, while RV performance improved, but did not reach pre-intervention levels. Cadherin-5, chitotriosidase-1, integrin-beta-2 and urokinase differed across time points and related to favorable RV GLS at T2.

Conclusions
Right, and to a lesser degree left, ventricular performance was reduced early after surgical ASD closure, possibly related to cardiopulmonary bypass. RV performance at T3 remained below baseline levels, probably related to changed loading conditions. Several biomarkers showed a pattern over time similar to ventricular performance and related to cardiac function at early follow-up. These markers may provide insight into the processes that affect ventricular function.
A large anterior mediastinal mass in a young infant
Authors: Davidson H , Veerappan S 
Published: February 2022

Abstract

Funding Acknowledgements
Type of funding sources: None.

TITLE
A large anterior mediastinal mass in a young infant

Case Report
A 4 week old male neonate, KS was referred to the emergency department with a 24 hour history of increased work of breathing, two brief episodes of apnoeas and inspiratory stridor. There was no infective symptoms or changes to his usual feeding pattern. There was no significant past medical history and he was on no regular medications. A chest x-ray demonstrated a large mediastinal mass and suspicion of cardiomegaly. A transthoracic echocardiogram demonstrated a structurally and functionally normal heart. However, there was a large anterior homogenous soft tissue mass extending to the left ventricular apex which was separate from the pericardium. This mass did not cause any heart chamber compression or compromise. Further cross-sectional imaging with cardiac computed tomography (CT) showed a non-enhancing uniform soft tissue mass occupying the superior and anterior mediastinum without evidence of calcification or fatty components. The lesion was seen to be extending from the root of the neck down to the left cardiac apex region with the total dimensions of the mass being 5.2 x 3.5 x 5cm. There was no compression of the great arteries or the airway. Parasternal ultrasound of the chest showed a starry sky appearance compatible with normal thymic tissue. Overall, the appearances were felt to be compatible with a prominent normal thymus.

Discussion
The differential diagnosis of an anterior mediastinal mass in young infants includes germ cell tumours (mainly teratomas), congenital thymic cysts and true thymic hyperplasia(1). These can be difficult to distinguish from the normal large neonatal thymus. The normal thymus can take on a variety of shapes and sizes and still be considered normal. One of the most important features of a normal thymus is the lack of mass effect or compression on the airway or vascular structures(2). True thymic hyperplasia is characterised by an increase in the size and shape of the thymus while preserving thymic architecture. If the anterior mass has a bipyramidal morphology with the presence of gross intercalated fat on CT, these findings are pathognomonic for thymic hyperplasia(3). Thymomas are rare in children and appear as well-defined, rounded or lobulated soft tissue density mass with mild enhancement on CT. It can demonstrate irregular borders with extension into adjacent structures and approximately 30% will have necrosis and internal cystic foci(4). The most common germ cell tumour in the mediastinum is a teratoma with the hallmark being fat, fluid and calcified components on CT(2). Lymphomas are the most common anterior mediastinal mass in children but are usually seen in children older than 5 years. They often cause mass effect with displacement of the trachea. CT scan demonstrates larger nodal areas with hypodense and cystic areas suggestive of necrosis(1). Abstract Figure 1 Abstract Figure 2