bluuebunny/crossref_metadata_2025_split huggingface dataset:
Development of psychopathology in adults with congenital heart disease: a 40–53 years follow-up study
Authors: Pelosi C , Kauling R M , Cuypers J A A E , Van Den Bosch A E , Bogers A J J C , Helbing W A , Roos-Hesselink J W , Legerstee J S , Utens E M W J 
Published: October 2022

Abstract

Background
Nowadays, more than 90% of patients with congenital heart disease (CHD) reach adulthood. However, little is known about long-term psychopathological problems in these patients.

Purpose
To investigate the development of psychological outcomes of adults with CHD operated in their childhood.

Material and methods
Our unique, single center, longitudinal cohort-study evaluates a series of consecutive patients who underwent cardiac surgery in childhood between 1968 and 1980 every 10 years. Standardized questionnaires were used to measure psychopathology at 4 follow-up time points (1991, 2001, 2011 and 2021) and outcomes were compared with normative data. Results in the current evaluation were corrected for the Covid-19 stringency index.

Results
At the current (4th) evaluation in 2021, 204 patients (46% female, age: 49.9±5.2) participated. Female patients with CHD reported significantly more internalizing problems than the normative group. More specifically, they reported significantly more somatic complaints. Proxy-reports showed significantly less externalizing problems for males. No significant difference was found between simple and moderate/complex CHD in terms of psychopathology. Correction for Covid-19 pandemic stringency showed no significant effect.
Over time, the percentage of patients scoring in the psychopathological range decreased from the first (1991, 25.4%) to the third follow-up (2011, 1.8%). However, the current study showed a significant increase (9.6%) of the psychopathology levels in comparison with 2011. (figure1)

Conclusions
Compared to normative data, female CHD patients reported significantly more internalizing problems, particularly more somatic complaints. Initially, the level of psychopathology decreased over time. However, in the last decade, there was a significant increase in psychopathology, not explained by the Covid-19 pandemic, warranting continuous attention to the psychological health of adults with CHD.

Funding Acknowledgement
Type of funding sources: Foundation. Main funding source(s): Thorax foundation
Extracellular volume fraction by computed tomography predicts long-term prognosis among patients with cardiac amyloidosis
Authors: Gama F , Rosmini S , Bandula S , Patel K P , Thornton G D , Bennett J B , Wechelakar A , Gillmore J D , Whelan C , Lachmann H , Taylor S , Fontana M , Moon J , Hawkins P N , Treibel T 
Published: October 2022

Abstract

Objective
This study sought to investigate the association of extracellular volume fraction by computed tomography (ECVCT), myocardial remodeling and mortality in patients with systemic amyloidosis.

Background
Light chain (AL) and transthyretin (ATTR) amyloid fibrils are deposited in the extracellular space of the myocardium, resulting in heart failure and premature mortality. Extracellular expansion can be quantified by CT, offering a rapid and cost-effective alternative to cardiovascular magnetic resonance (CMR), especially among patients with cardiac devices or on renal dialysis.

Methods
Patients with confirmed systemic amyloidosis and varying degrees of cardiac involvement underwent ECG-gated cardiac CT. ECVCT was analysed in the inter-ventricular septum. All patients also underwent clinical assessment, ECG, echocardiography, serum amyloid protein component (SAP) and/or technetium-99m (99mTc) 3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy. ECVCT was compared across different extents of cardiac infiltration (ATTR Perugini Grade / AL Mayo Class) and evaluated for its association with myocardial remodelling and all-cause mortality.

Results
72 patients were studied (AL n=35, ATTR n=37; age 67 (59–76) years, 71% males). Mean septal ECVCT was 42.7±13.1% and 55.8±10.9% in AL and ATTR, respectively, and correlated with indexed left ventricular (LV) mass (r=0.426, p&lt;0.001), LV ejection fraction [LVEF, (r=0.460, p&lt;0.001)], NT-proBNP (r=0.563, p&lt;0.001) and hsTnT (r=0.546, p=0.02). ECVCT increased with cardiac amyloid involvement in both AL and ATTR (Figure 1). Over a mean follow-up of 5.3±2.4 years, 40 deaths occurred (AL 14 [35%]; ATTR 26 [65%]). ECVCT was independently associated with all-cause mortality in ATTR (not AL) after adjustment for age and IV septal wall thickness (HR: 1.046, 95% CI: 1.003–1.090, p=0.037).

Conclusion
Cardiac amyloid burden quantified by ECVCT is associated with adverse cardiac remodelling as well as all-cause mortality among ATTR amyloid patients. ECVCT may address the need for better identification and risk stratification of amyloid patients, using a widely-accessible imaging modality (Figure 2).

Funding Acknowledgement
Type of funding sources: None.
Predictors of of arrythmia in adult patients with Ebstein anomaly
Authors: Wals Rodriguez A J , Gallego P , Pijuan Domenech A , Rodriguez Puras M J , Miranda Barrio B , Gordon Ramirez B , Ladron Abia R , Gonzalez Fernandez V , Dos Subira L 
Published: October 2022

Abstract

Objectives
The objective of this study was to evaluate the incidence and risk factors for arrhyhthmias in adult patients with Ebstein anomaly (EA).

Methods
Retrospective observational study of a multicenter cohort of 123 adult patients with EA. Demographic, clinical and echocardiography data at their first visit at the Adult Congenital Heart Disease Unit were collected. Free-event survival was assessed with Kaplan Meier curves and differences between groups using the log-rank test. Predictors of arrythmia were assessed using Cox regression analysis.

Results
Median follow up (FU) was 6 (IQ 2–12) years and the median age at the end of FU was 47 (IQ 33–60) years. 61.8% (n=76) were women and 68 patients (55%) had associated atrial septal defects. A total of 43 patients (34%) were repaired and 11 (8.9%) required reoperation. During FU 40 patients (32.5%) had supraventricular arrhythmias (SA), mainly atrial flutter (n=19; 15.6%) and atrial fibrillation (n=14; 11.5%); 12 patients (9.8%) developed high-grade atrio-ventricular block (AVB). In the multivariate analysis, the baseline variables associated with the appearance of SA were history of cyanosis (HR: 2.269 (1.104–4.666); p=0.026) and NYHA functional class ≥ II at baseline (HR: 2.620 (CI: 1.062–6.465); p=0.037). For high-grade AVB, the associated variable was tricuspid valve implant during FU (HR: 7.993 (CI: 1.396–45.770); p=0.020).

Conclusions
Arrhythmias, particularly SA, are a common complication in adult patients with EA. Patients with a NYHA functional class ≥ II at baseline and those exposed to cyanosis are more prone to develop SA during FU, whereas patients requiring tricuspid valve implant are at increased risk of developing high-degree AVB.

Funding Acknowledgement
Type of funding sources: Public hospital(s). Main funding source(s): Hospital Vall d'HebronHospital Univertsitario Virgen del Rocío
Predictors of long term tricuspid regurgitation after pulmonary valve replacement: a single center study on the cohort of significant preoperative tricuspid regurgitation
Authors: Carbonell Prat B , Dos L , Gordon B , Ligero C , Gonzalez V , Vilte G , Pijuan A , Miranda B , Ferre M , Lorente M , Guedes P , Ferreira I , Castro M A 
Published: October 2022

Abstract

Background
Almost one-third of repaired tetralogy of Fallot (TOF) and pulmonary stenosis (PS) patients referred for pulmonary valve replacement (PVR) have at least moderate tricuspid regurgitation (TR). Predictors of long term TR in this population are not well defined and the role of Tricuspid Annuloplasty (TA) in addition to PVR in this setting is controversial.

Purpose
Patients with preoperative severe TR or moderate TR with dilated tricuspid annulus (&gt;40mm) undergo TA at the time of PVR as per protocol in our instititution. The aim of this observational study was to evaluate the long term results of this approach and to identify predictors of significant TR at the end of follow-up.

Material and methods
We retrospectively reviewed 35 repaired TOF and PS patients who underwent surgical PVR with (n=17, group 1) or without (n=18, group 2) TA in a single tertiary hospital between June-2009 and June-2021. Pre- and postoperative echocardiographic and cardiac magnetic resonance data were assessed for TR severity and right ventricle size and systolic function. Baseline and clinical follow up data were collected. Uni- and multivariable tests were used to analyze predictors of at least moderate TR after PVR.

Results
Mean age at PVR and baseline image parameters were comparable between groups, except for TR grade (p 0.006), tricuspid annulus size (p 0.018) and right ventricle outflow tract diameter (p 0.04) which were greater in group 1. TA did not confer and increased risk of surgical and peroperative complications. Intensive care unit (p 0.94) and total hospital admission days (p 0.59) did not differ among groups. At one year, TR grade significantly improved regardless of TA. At the end of follow-up (5.29±2.9 years), the degree of TR increased in both groups with no differences between groups (p 0.38) (see table 1). In univariable analysis, two independent predictors of at least moderate TR at end of follow-up were identified; mixed TR mechanism (OR 12.5, p 0.006) and severe preoperative TR grade (OR 10.21, p 0.003). Mixed TR mechanism (OR 15.78, p 0.01) and a right ventricle ejection fraction below 45% (OR 1.47, p 0.01) were identified as risk factors of significant TR in multivariable analysis (see Table 2).

Conclusions
TA seems a reasonable approach at the time of PVR in patients with severe TR or moderate TR with dilated annulus. Mixed TR mechanism and a worse right ventricular function have been identified as independent predictors factors of long term significant postoperative TR in our study cohort.

Funding Acknowledgement
Type of funding sources: None.
Impact of coronavirus disease 2019 on adult patients with congenital heart disease
Authors: Cardoso I , Branco T , Constante A , Viegas J , Grazina A , Teixeira B , Jacinto S , Castelo A , Rito T , Martins D , Ferreira R , Sousa L 
Published: October 2022

Abstract

Background
COVID-19 is responsible for a worldwide pandemic, causing more than 18,000 deaths to date in Portugal. Data already exists regarding the increased risk of adverse events in patients with cardiovascular diseases, however the impact of SARS-CoV-2 infection in patients (P) with congenital heart disease (CHD) is still under investigation.

Purpose
To study the impact of COVID-19 in a adult patients with CHD

Methods
Adult patients seen at the CHD outpatient's clinic at a tertiary centre, who became infected with SARS-CoV-2 infection up to December 2021 were included. Assessment of patients' symptoms, need for hospitalization and admission in an intensive care unit was assessed based on medical records.

Results
We identified seventy-nine patients (pts) with COVID-19 infection. Symptoms were present in 67 (84%). The median age was 44 (15) years, 52% were females. Eight P (10%) had complex cyanotic disease; seven Tetralogy of Fallot; five (6%) transposition of great arteries; eight (10%) right ventricle obstacle; two (3%) atrioventricular canal defect; sixteen (20%) atrial septal defect; nine (11%) ventricular septal defect; eight (10%) aortic coarctation; two (3%) had Eisenmenger syndrome. 49% of P had previous surgery or percutaneous procedure. 63% of P were at New York Heart Association (NYHA) class of I and 30% at NYHA II. Mild symptoms were reported by 56 P (71%). Ten adults (7,9%) experienced moderate symptoms (dyspnea and hypoxia) that led to hospitalization for oxygen therapy, none required mechanical ventilation. One death was reported in an 83-year-old patient with non-corrected interventricular communication and compromised biventricular function. There was a significant association between the gravity of CHD and hospitalizations (p=0.02).

Conclusion
Our pts had mainly mild to moderate symptoms and did not appear to have a disproportionately negative outcome; the need for hospitalization was more frequent in patients with higher CHD gravity. These findings are in line with the emerging data regarding COVID-19 in CHD P, and may be in part explained by the patient's young age and functional status.

Funding Acknowledgement
Type of funding sources: None.
Human versus machine: does artificial intelligence add value to identification of hypertrophic cardiomyopathy in pediatric patients?
Authors: Guerrier K , Gunturkun F , Wetzel G , Akbilgic O , Davis R , Towbin J 
Published: October 2022

Abstract

Introduction
An abnormal increase in left ventricular thickness is a hallmark of hypertrophic cardiomyopathy (HCM). Although standard measures of left ventricular voltage abnormalities on electrocardiograms (ECGs) have high false positive rates and poor correlation to left ventricular thickness, ECGs continue to be part of most screening programs. We developed a machine learning model for HCM classification from ECG in a pediatric cohort and compared its efficacy to that of clinician specialists.

Purpose
To compare clinician-based predictions of HCM to a machine learning model trained to classify pediatric patients with HCM utilizing 12-lead ECG.

Methods
ECGs from patients &lt;19 years with HCM, including those with HCM gene mutations, were compared to those from age- and sex-matched controls with normal heart structure and function on echocardiogram. Patients with a known history of primary causes of left ventricular hypertrophy such as aortic stenosis or glycogen storage disease were excluded. A cascaded convolutional neural network was developed combining a residual neural network with a 2-layer dense neural network with 10-fold cross validation. The performance of the machine learning based HCM classification was compared to that of two independent clinicians.

Results
Analytic sample included data from 82 patients with clinical HCM compared to 91 healthy control subjects. The machine model AUC was 0.89 (0.84–0.94). Clinician inter-rater reliability was 0.8. The clinicians had a higher specificity (97% vs 82%) but lower sensitivity (50% vs 78%) than the machine learning model. Compared to clinician-classification, the positive predictive value was lower in the machine model (82% vs 93%).

Conclusion
In this preliminary study, machine learning classification of HCM utilizing 12-lead ECG had greater sensitivity than that of clinician interpretation. Machine learning may play a role in screening and diagnosis, including in subjects with normal-appearing ECG. Evaluation of its utility in a larger cohort is needed.

Funding Acknowledgement
Type of funding sources: Public hospital(s). Main funding source(s): Le Bonheur Children's Hospital
The association between age, troponin level, and mortality in patients hospitalised with acute pulmonary embolism (NIHR Health Informatics Collaborative TROP-PE study)
Authors: Kaura A , Goswami S , Mulla A , Glampson B , Davies J , Woods K , Shah A M , Kharbanda R , Patel R S , Perera D , Channon K M , Quint J , Mayet J 
Published: October 2022

Abstract

Background
A positive cardiac troponin (cTn) is an independent predictor of short-term mortality in individuals presenting with acute pulmonary embolism (PE). However, there is limited evidence regarding the impact age has on the association between cTn levels and mortality in patients with PE.

Purpose
The aim of our study was to investigate the relationship between cTn level, age, and all-cause mortality, in hospitalised patients diagnosed with an acute PE.

Methods
A retrospective cohort study using the National Institute for Health Research Health Informatics Collaborative Cardiovascular dataset of all consecutive patients who had a troponin measured at five hospitals between 2010 and 2017. Patients admitted to hospital with a primary diagnosis of PE with at least one cTn measurement were included. We modelled the relation between peak troponin level and all-cause mortality using multivariable adjusted restricted cubic spline Cox regression analyses. Effect estimates were adjusted for age, gender, high-sensitivity troponin assay, C-reactive protein, haemoglobin, platelet count, white cell count, creatinine, sodium, potassium, diabetes, hypertension, hypercholesterolaemia, acute coronary syndrome, atrial fibrillation, heart failure, acute kidney injury, chronic kidney disease, obstructive lung disease, inflammatory disorders, pneumonia and malignancy. The peak cTn level (highest level measured), standardised to the upper limit of normal (ULN), was used for all analyses.

Results
1,477 patients with at least one cTn measurement and a diagnosis of acute PE were included. During a median follow-up of 34.8 months, there were 290 (19.6%) deaths. Elevated cTn (&gt;1xULN) was associated with a hazard ratio (HR) of 3.29 (95% confidence interval [CI] 1.95–5.53) for 30-day mortality and 2.12 (95% CI 1.63–2.75) for 3-year mortality. Higher cTn levels were progressively associated with a higher mortality risk, reaching a maximum HR of 2.59 (95% CI 1.64–4.09) at 141xULN (Figure 1). Younger patients (&lt;55 years), compared with those aged over 55, had the highest 3-year HR associated with a positive cTn of 2.94 (95% CI 1.48–5.82) despite having the lowest troponin levels (mean 7.01xULN) on admission (Figure 2).

Conclusion
Elevated cTn, at all ages, is associated with an increased mortality risk in patients presenting with PE, with increasing cTn levels conferring a progressively worse long-term prognosis. Elevated cTn, no matter how small, needs to be taken seriously, particularly in young patients with an acute PE.

Funding Acknowledgement
Type of funding sources: Public grant(s) – National budget only. Main funding source(s): 1) NIHR Imperial Biomedical Research Centre, as part of the NIHR Health Informatics Collaborative, and 2) British Heart Foundation
Paediatric pulmonary arterial hypertension following congenital heart defect repair: enhanced risk stratification and outcomes in a national cohort
Authors: Constantine A , Dimopoulos K , Condliffe R , Clift P , Jansen K , Dhillon R , Chaplin G , Muthurangu V , Moledina S 
Published: October 2022

Abstract

Introduction
Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD). PAH following CHD repair (repaired PAH-CHD) is an emerging population with increased morbidity and mortality [1–3]. Risk scores that are widely used in the adult PAH population have not been validated and may not be applicable in young children with repaired PAH-CHD.

Purpose
To characterize the 20-year cohort of the UK National Paediatric PH Service, focusing on peri-operative characteristics and outcomes of children with repaired PAH-CHD, and develop a tailored risk stratification tool.

Methods
We included consecutive children presenting to our specialist service between 2001 and 2021 with a diagnosis of repaired PAH-CHD. Patients with univentricular physiology, segmental pulmonary hypertension (PH), and PH primarily due to abnormal development of the pulmonary vasculature were excluded. Univariable Cox regression analysis was performed in children with PAH present ≥3 months after CHD repair, with an outcome of death or transplantation. Time-dependent ROC analysis was used to evaluate the risk model.

Results
Overall, 178 patients were included (age 3.2 [1.3–7.9] years, 58.4% female). Most children (73.0%) were referred following CHD repair. Complex CHD was present in 61.2%, and 48.9% had combined pre- and post-tricuspid shunts. Down syndrome was present in 33.1%. At the first post-operative PH assessment, 53.1% of patients had symptoms, mainly breathlessness. On echocardiographic evaluation, 30.9% had moderate-severe right ventricular dilatation and 23.7% had right ventricular systolic impairment. Over a median follow-up of 6.0 [2.4–11.0] years, 30 (19.2%) patients died and 5 (3.2%) patients underwent lung transplantation. Transpant-free survival at 1, 5, and 10 years was 94.7% (95% CI: 91.2–98.3%), 85.9% (95% CI: 80.3–91.9%), and 80.1% (95% CI: 73.1–87.7%), respectively. Cox analysis identified several clinical variables associated with death or transplantation, of which the following variables assessed post-repair were used to develop the risk score: absence of pre-operative PH, breathlessness, right ventricular dysfunction, and pulmonary vascular resistance index &gt;14 WU m2 or lesion at high risk of causing PH (Figure 1). This novel, simple risk score performed well (AUC &gt;80% at 1, 3, 5 and 10 years) and was well-calibrated in our cohort (Figure 2, D'Agostino-Nam p=0.76).

Conclusions
In this national cohort of children with repaired PAH-CHD, mortality is significant. This novel, simple risk score performed well in our population and could be used in clinical practice at the time of post-operative assessment to predict outcome and direct management but requires further validation.

Funding Acknowledgement
Type of funding sources: None.
Increased risk of long-term mortality following pulmonary embolism in Denmark, 2000–2020: a nationwide trend study
Authors: Fredslund Madsen S , Moelager Christensen D , Strange J E , Nourhavesh N , Kumler T , Gislason G , Lamberts M , Sindet-Pedersen C 
Published: October 2022

Abstract

Background
New diagnostics and treatment regimens have been introduced in the last 20 years which may have influenced the prognosis of pulmonary embolism (PE). However, there is a lack of studies investigating temporal trend in short- and long-term mortality following PE in different subgroups.

Purpose
We aimed to investigate temporal trends in short- and long-term mortality following PE according to age, sex, and cancer status.

Method
This study was based on data from Danish national registries. All patients with a first-time inpatient hospital diagnosis of PE between the 1st of January 2000 through 3rd of December 2020 were included in the study. Mortality was calculated as the proportion of patients who died within 30 days and 31–365 days, respectively, and were presented with 95% confidence intervals (CI), and additionally stratified into categories of age groups (18–39, 40–59, 60–79, and 80–100 years), sex (male/female) and cancer status (no cancer/cancer). Cancer was defined as a cancer diagnosis registered within 6 months prior to the PE diagnosis.

Results
In total 62,750 patients had a first-time PE during the study period. The mean age of the study population was 68.3 years (sd), and 32,617 (51.9%) were women and 18,719 (29.83%) of patients had cancer. The 30-day mortality for age groups, sex, and cancer status decreased during the study period 2000–2020 (Figure 1A). The 31–365-day mortality for age group 40–59 years increased from 5.7% [95% CI: 3.7–8.5] in 2000 to 9.5% [95% CI: 7.5–11.9] in 2019. Age group 80–100 years increased from 18.5% [95% CI: 14.8–22.8] in 2000 to 23.5% [95% CI: 21.0–26.2] in 2019. No temporal differences were observed for age groups 18–39 and 60–79 years. Mortality for cancer-associated PE increased from 24.8% [95% CI: 19.8–30.6] in 2000 to 29.9% [95% CI: 27.4–32.4] in 2019 (Figure 1B).

Conclusion
Despite overall mortality following PE has improved, our data suggest that short-term mortality following pulmonary embolism has improved considerably while long-term mortality were increased. Future studies should assess whether this phenomenon is due to a different patient risk profile (e.g. cancer) or lack of longer term care (e.g. rehabilitation program, withdrawal of antithrombotic treatment).

Funding Acknowledgement
Type of funding sources: None.