bluuebunny/crossref_metadata_2025_split huggingface dataset:
Long-term outcomes in patients with dual pathology aortic stenosis and cardiac amyloidosis referred for valve intervention
Authors: Patel K , Autherith M , Scully P , Koschutnik M , Dona C , Halavina K , Kammerlander A , Menezes L , Pugliese F , Fontana M , Lloyd G , Mascherbauer J , Moon J , Nitsche C , Treibel T 
Published: October 2024

Abstract

Background
The coexistence of aortic stenosis (AS) and cardiac amyloidosis (CA) is common. If treated with transcatheter aortic valve implantation (TAVI), patients with the combined phenotype (AS-CA) have a comparable short term survival to those with lone AS, whereas data on longer term outcomes is currently lacking.

Purpose
This study aimed to evaluate the clinical outcomes of AS-CA compared to lone AS over a 5-year follow-up.

Methods
Using a prospective, multicentre, observational, case-control design, we screened consecutive patients with severe AS referred for TAVI for co-existing CA. CA screening included blinded 99mTc-DPD bone scintigraphy (Perugini Grade-0 negative, 1-3 increasingly positive) with additional SPECT/CT and light chain assessment prior to intervention. Transthyretin CA (ATTR) was diagnosed by bone scintigraphy and unremarkable light chain assessment, light-chain CA (AL) by endomyocardial biopsy. Mortality (all-cause and cardiovascular [CV]) and hospitalisation for heart failure (HHF) were captured as clinical endpoints.

Results
376 patients (83±7 years, 52% female, EuroSCORE-II 4.9±2.9) were recruited, of which 43 (11.4%) had AS-CA (42 ATTR, 1 AL). Compared to lone AS, patients with AS-CA were older with higher cardiac biomarkers (NT-proBNP, high-sensitive Troponin-T) and a higher prevalence of atrial fibrillation. Heart team decision yielded valve replacement in 320 (85%) and conservative management in 56 (15%) patients, without differences between AS-CA and lone AS.
Over a median follow-up of 5.4 (Q1: 4.9; Q3: 5.7) years, 230 (61.1%) patients died and 67 (17.8%) experienced HHF (with a total HHF number of 91). AS-CA was associated with higher all-cause mortality (crude HR 1.64, 95%CI 1.15-2.35; log-rank, p=0.006), which remained significant after multivariate adjustment for clinical confounders (EuroSCORE-II, valve replacement; adjusted HR 1.63, 95%CI 1.15-2.32; p=0.006). AS-CA was not associated with CV mortality (log-rank, p=0.18) or time to first HHF (log-rank, p=0.43), but the rate of HHF was significantly higher in AS-CA compared to lone AS (5.7 versus 3.5 per 1,000 patient years, p=0.022).

Conclusions
Among elderly patients referred for TAVI, long-term outcomes of AS-CA are characterized by higher mortality and a higher rate of heart failure hospitalisations compared to patients with lone AS. Studies evaluating the role of CA-specific treatments are warranted in this population.
Prognostic staging system correctly identifies high risk groups in cardiac transthyretin amyloidosis treated with tafamidis
Authors: Mueller M , Brand A , Mattig I , Spethmann S , Messroghli D , Hahn K , Landmesser U , Heidecker B 
Published: October 2024

Abstract

Background
The development of different multiparametric staging systems improved the risk assessment of cardiac transthyretin amyloidosis (ATTR-CA) patients substantially. However, to date, all staging systems were validated in treatment-naive ATTR-CA patients. The introduction of tafamidis led to significant changes in the disease trajectory of ATTR-CA, indicating that it may also have an impact on the prognostic accuracy of these staging systems.

Purpose
Thus, we sought to assess whether the prognostic value of the National Amyloidosis Centre (NAC) staging system, currently considered as the most accurate, is sustained in ATTR-CA patients treated with tafamidis.

Methods
This retrospective observational study included ATTR-CA patients treated with tafamidis. Patients were continuously followed from treatment initiation to time of death. NT-proBNP and eGFR data collected at baseline were used to stratify patients into low (stage I), intermediate (stage II) and high-risk (stage III) subgroups according to the NAC staging system. Kaplan-Meier analyses were conducted to illustrate overall survival. Differences in overall survival between subgroups were assessed by log rank tests. P-values &lt;0.05 were considered statistically significant.

Results
A total of 166 ATTR-CA patients (95.2% wild-type) were enrolled. 81 (48.8%), 51 (30.7%) and 34 (20.5%) patients were stratified into stages I, II and III, respectively. Median follow-up was 539 [323-865] days, during which 20 deaths were recorded. Overall survival of the subgroups over time is demonstrated in figure 1. The probability of overall survival was significantly lower for patients in stage III compared to patients in stages I (log rank; p=0.002) and II (log rank; p=0.031). However, no differences in the probability of overall survival were identified between patients in stage I and II (log rank; p=0.679).

Conclusions
In this cohort of ATTR-CA patients treated with tafamidis, the NAC staging system effectively identified patients at elevated risk of mortality. However, the distinction in survival rates between low- and intermediate-risk groups was less pronounced, suggesting that tafamidis may have a stronger prognostic impact in these groups.Overall Survival across NAC Stages
ATTR cardiac amyloidosis after lumbar spinal stenosis- a prospective cohort study
Authors: Eldhagen P , Tzortzakakis A , Sorensson P , Lund L H , Westermark P , Soderstrom L , Berg S 
Published: October 2024

Abstract

Background
Systemic wild type transthyretin (ATTRwt) amyloidosis is underdiagnosed and generally diagnosed in manifest cardiac disease. Lumbar spinal stenosis (LSS) might be an early sign of ATTRwt and a possible screening target for early diagnosis.

Aim
To characterize patients with transthyretin amyloid deposits in ligamentum flavum six years after surgery for lumbar spinal stenosis, and assess prevalence of undiagnosed cardiac amyloidosis.

Materials and methods
In this prospective cohort study, 21 patients who had surgery for LSS in 2016-2018 and grade 3-4 ATTR deposits in ligamentum flavum, without manifest cardiac amyloidosis (CA) were followed up in 2022-2023, including biomarkers and multimodality cardiac imaging.

Results
Median age at follow-up was 79 years and 62% were male; 16% (3/19) of patients had DPD-scintigraphy and were diagnosed with ATTRwt cardiac amyloidosis. 48% (10/21) had a history of other tenosynovial conditions that associate to ATTRwt amyloidosis. on echocardiography.

Conclusion
In this prospective cohort study, 16% of patients were diagnosed with ATTRwt cardiomyopathy after a median of six years following surgery for lumbar spinal stenosis. These findings strengthen the hypothesis that LSS is a possible manifestation of ATTRwt amyloidosis and that analysis for amyloid in connective tissue and subsequent cardiac follow-up is of value.
A novel risk-stratification model that predicts long-term clinical outcomes in patients with recurrent pericarditis
Authors: Kumar A , Abdullah Yesilyaprak A Y , Ankit Agrawal A A , Beni Verma B V , Muhammad Furqan M F , Alveena B Syed A B S , Muhammad Majid M M , Danny L Rayes D L R , Tom Kai Ming Wang T K M , David Chen D C , Paul C Cremer P C , Allan L Klein A L K 
Published: October 2024

Abstract

Background
Recurrent Pericarditis (RP) is condition associated with significant debility and morbidity. Previous studies have identified variables such as elevated C-Reactive protein, corticosteroid usage, and presence of delayed hyper-enhancement (DHE) on cardiac magnetic resonance imaging are associated with recurrence. To date however, there is no established model for risk stratifying outcomes in these patients, therefore we attempted to develop a model that could predict long-term outcomes in patients with RP and stratify them into groups based on risk.

Methods
A total of 365 consecutive patients with RP from 2012-2019 were retrospectively examined. Our primary outcome was clinical remission (CR) defined as symptom resolution in addition to complete cessation of all anti-inflammatory therapy. We employed 5 survival models: CoxPH, RSF, SSVM, GBSA and XGBoost with Cox loss function to determine the likelihood of achieving CR within 5 years and to stratify patients into specific risk groups. We divided the dataset into training (70%) and test (30%) sets. Model training and optimization of the training set occurred via 5-fold cross validation. Model evaluation and selection was based on C-index. Finally, we employed an explainable artificial intelligence approach, SHapley Additive exPlanations to generate both individual and global explanation of the models decision.

Results
Amongst the cohort, the mean age was 46 ± 15 years, 205 (56%) were female, and the main etiology was idiopathic in 223 (61%). CR was achieved in 118 (32%) patients. The final model included steroid dependency, total number of recurrences, pericardial DHE, age, etiology, gender, ejection fraction and heart rate as its most important parameters. We observed similar CR rates across three risk score ranges and accordingly stratified patients into low (n=62), intermediate (n=154), and high-risk (n=149) groups. Scores of 3 or lower were categorized as low risk, with CR rates exceeding 90%. Scores of 4 to 7 were associated with CR rates of 50%. Scores of 8 or higher were deemed high-risk, with CR rates notably below 10%. Overall, the model predicted the outcome with C-index of 0.800 on the test set and exhibited a significant ability in stratification of patients into low-, intermediate-, and high-risk groups (log-rank test; P &lt;0.0001).

Conclusion
We created a novel risk prediction model that can aid in predicting rates clinical remission in patients with recurrent pericarditis as well as aid in stratification into low, medium, and high risk groups. This risk prediction model may be helpful in diagnosis and management of these complicated patients.Figure 1
Short-pulse of systemic steroids in acute pericarditis: an alternative in refractory cases
Authors: Sanchez Arjona A , Fernando Dominguez Rodriguez F D R , Juan Carlos Lopez Azor J C L A , Marta Cobo Marcos M C M , Gabriela Uriarte Zabala G U Z , Alejandro Nistal Juarez A N J , Pablo Garcia Pavia P G P , Javier Segovia Cubero J S C 
Published: October 2024

Abstract

Introduction
Acute pericarditis accounts for 5% of emergency room admissions for chest pain. First line treatment consists of non-steroidal anti-inflammatory drugs (NSAIDs) and colchicine. Systemic corticosteroids (SCS) are usually reserved as a second-line therapy due to a higher rate of recurrences and side effects. Whether a short pulse of low-dose SCS is safe and effective during the acute phase of the disease remains unknown.

Purpose
To investigate safety and efficacy of a 3 day pulse of 40 mg intravenous methylprednisolone in patients hospitalized for acute pericarditis with a lack of response to NSAIDs and colchicine.

Methods
A retrospective, observational and unicentric study was performed including patients hospitalized for acute pericarditis between 2019 and 2023. Patients with asymptomatic pericardial effusion or predominance of myocardial injury were excluded. Patients were considered to be refractory to conventional treatment when at least one of the following criteria was present despite five days of high-dose NSAIDs and colchicine: 1- Moderate/severe pericardial effusion persistence. 2- Chest pain persistence. 3- Increase of inflammatory biomarkers. These patients received methylprednisolone 40mg for three days on top of standard treatment. Baseline characteristics, treatment received during hospitalization and events during follow-up were analysed. Data were expressed as mean and standard deviation (quantitative variables) and percentages (categorical variables). Two-sided P value of less than 0.05 was considered statistically significant.

Results
70 patients with acute pericarditis requiring hospitalization were included (54±19 years; 71% male) with a mean follow-up of 10±21 months. 26 of them (37%) received methylprednisolone 40mg for three days due to lack of response to standard treatment. Table 1 includes a comparison of patients who received systemic corticosteroids versus those who did not (control group). C-reactive protein (CRP) was significantly higher in the group receiving corticosteroids (188±86 mg/L vs 135±85 mg/L; p=0.014) and it significantly decreased during hospital stay compared to the control group (151±87 mg/L vs 97±74 mg/L; p=0.013). 14 patients (20%) experienced a recurrence of chest pain during follow-up with no significant difference according to the treatment received (27% vs 16%; p=0.266)

Conclusion
A 3-day pulse of low-dose SCS seems to be useful and safe for hospitalized patients with acute pericarditis who show lack of response to NSAIDs and colchicine, with no increase in recurrence rate. However, more evidence based on randomized control trials is needed to support this therapeutic approach.Table 1
Heart failure burden among adults with congenital heart disease
Authors: Benderly M , Zureik M 
Published: October 2024

Abstract

Background
Adult congenital heart disease (ACHD) is associated with high rates of healthcare utilization and mortality. Data on Heart failure (HF) burden among ACHD patients are limited.

Purpose
To study the association between HF and healthcare utilization and mortality among ACHD patients in the community.

Methods
The retrospective cohort study comprised 11,653≥ 18 year old ACHD patients insured by two large Israeli healthcare providers between January 2007 and December 2011. We used multivariable Cox proportional hazard and negative binomial models to study the associations of HF with mortality and healthcare utilization, respectively. Inverse treatment probability weighting accounted for drug indication.

Results
ACHD-HF patients (N=908; 7.8%) were older (median: 69 vs. 45 years), more likely men (53% vs. 45%), with complex congenital heart disease (14% vs. 4%), and higher comorbidity rates than ACHD patients without HF. HF was associated with higher healthcare utilization. Compared to patients without HF, ACHD-HF patients had higher adjusted relative rates (RR) of primary care (1.26, 95% confidence interval [CI]: 1.17-1.3), and cardiology clinic visits (1.9, 95% CI: 1.7-2.0), higher hospitalization rates (RR=2.0, 95% CI: 1.7-2.1), and higher risk of death (mortality hazard ratio [HR]: 1.9, 95% CI: 1.5-20.) over the study period. Among ACHD-HF patients, Angiotensin-converting enzyme inhibitors or angiotensin receptor blockers, beta-blockers, and diuretics were associated with a lower mortality risk (treated vs. untreated HR=0.56, 95% CI: 0.48-0.65; HR=0.7, 95% CI: 0.6-0.8; HR=0.84, 95% CI: 0.73-0.97; respectively).

Conclusions
HF presents a major burden on ACHD patients and health care systems. Drugs recommended to the general HF patient population are also associated with lower risk of hospitalization and mortality in ACHD patients. Clinical trials are needed to establish the benefit of HF pharmacotherapy in ACHD patients.Age adjusted ACHD patient survival by HF
Transcatheter closure of atrial septal defect (ASD) in adult and paediatric population without transoesophageal echocardiography/general anaesthesia
Authors: Barwad P , Naganur S , Sharma Y P , Mahajan S 
Published: October 2024

Abstract

Background
Transcatheter device closure is the preferred therapeutic option for ostium secundum atrial septal defect(OS-ASD). Standard practice is to perform it under transoesophageal echocardiography(TEE) guidance. However, TEE is performed under general anaesthesia (GA) and leads to increases in cost, procedural duration, ICU stay, total hospital stays, risk of oesophageal injury and complications of GA. Transthoracic echocardiography(TTE) is an excellent alternative and prior studies have shown it to be a safe alternative in paediatric population. In current study we analysed safety and efficacy of TTE-guided device closure of OS-ASD in all comer population irrespective of age, weight, defect size.

Methods
The study enrolled all patients who underwent device closure of OS-ASD between March 2017 and December 2023. All procedures were performed under LA for adults and conscious sedation for children. The device was placed under fluoroscopy guidance and position were confirmed on TTE. Procedural success, device, defect size ratio, procedural time, fluoroscopy time, complications, intensive care unit/hospital stay duration were analysed and compared with our own experience of 125 patients with TEE-guided closures before switching to complete TTE-guided procedure(before 2017).

Results
340 patients underwent TTE-guided device closure of ASD. Procedure success was 97.4% with acceptable risk of major complications. Three(0.8%) had embolization into pulmonary artery, successfully operated surgically. Two had tamponade requiring drainage. In 9 patients, device could not be deployed despite multiple attempts and approaches. All of these had defect size of &gt; 34mm and deficient IVC rim along with shorter (other than aortic) rim. Findings were compared with our own previous experience of TOE guided closure (practised before 2017). Compared to TOE group, TTE group had significantly lower total procedural duration (75±4 vs. 28 ±1 min; p &lt; 0.01), intensive care unit stay (24±10 vs. 10.5±1 h; p &lt; 0.01), and hospital (48±12 vs. 25.5±4h; p &lt; 0.01) stay duration. Fluoroscopic time was similar in both. There was no mortality in either group. Major complications were comparable, TTE-guided group had less post-procedure complications of vomiting and headache. Additional findings of less pre-procedure fasting duration and early commencement of post procedure oral intake were noted in TTE group.

Conclusion
TTE-guided device closure of OS-ASD is a safe and feasible in all-comer population in experienced hands. It can definitely negate the use of TEE except in very poor echo window. The added advantages include utilization of physiological variations in PV/LV pressure during cough, Valsalva manoeuvres in awake patients. It definitely reduces the procedural duration, cost of procedure and dependence on GA and trained transoesophageal echocardiographer.
The incidence of atrial fibrillation in patients with interatrial shunt: is it reduced after surgical or transcatheter closure of the shunt?
Authors: Karagianni A , Eriksson P , Papadopoulos S , Dellborg M , Mandalenakis Z 
Published: October 2024

Abstract

Background
Atrial fibrillation (AF) is a prevalent diagnosis among individuals with interatrial shunts, and the prevalence increases with age (1). Since the 1990s, interventionists have introduced transcatheter techniques, to close interatrial shunts, while surgical procedures reserved in cases of technical complexity (2). Despite the potential reduction in AF incidence post interatrial shunt closure, discernible modifications in the cardiac conduction system may persist (3). Notwithstanding, reports indicate an incidence of new-onset AF in approximately 10-25% of patients after interatrial shunt closure, with a pronounced prevalence among elderly (4). This study aims to delineate the incidence of AF subsequent to interatrial shunt, surgical och transcatheter, closure within a national cohort in Sweden.

Methods
The study includes all patients diagnosed with an interatrial shunt, atrial septal defect and patent foramen ovale, classified using the International Classification of Diseases, 10th edition (ICD-10), as documented in the Swedish National Patient Register, along with data sourced from the Cause of Death Register. Patients with multiple diagnoses of other congenital heart diseases are excluded, and interventions performed between 1997 and 2017 are identified using the NOMESCO surgical classification. Comorbidities such as chronic ischemic heart disease, myocardial infarction, heart failure, hypertension, and diabetes are considered, with the primary endpoint being the incidence of AF post-intervention.

Results
A total of 3,426 patients with interatrial shunts underwent intervention between 1997 and 2017, with a mean follow-up duration of 6.2 years. Among these, 49% (n=1,725) underwent transcatheter closure, while 41 patients underwent both transcatheter and surgical procedures. The mean age was 36 years, with 9% presenting with heart failure at baseline. Before the intervention, 14% (n=483) had AF, increasing to 22% (n=755) post-intervention. Of those developing AF post-intervention, 16% (n=119) experienced the AF event within 60 days, evenly distributed between transcatheter and surgical procedures. Over 40% of patients with post-intervention AF had an average age of 62. The incidence of AF post-intervention was 1.6 per 100 patient-years (see Figure). Stroke incidence was 3.6 per 100 patient-years and the mortality rate was 7% (n=257) during follow up (see Table).

Conclusions
Patients with interatrial shunts exhibit a high incidence of AF, which further escalates following closure of the interatrial shunt, not only within the first few days post-intervention, particularly among the elderly population. This can relate to high incidence of ischemic stroke.Figure
A novel risk score based on exercise capacity to predict all-cause mortality in adults with congenital heart disease
Authors: Skogby S , Wiklund U , Wikner A , Rinnstrom D , Christersson C , Sorensson P , Trzebiatowska-Krzynska A , Mandalenakis Z , Hlebowicz J , Sandberg C , Johansson B 
Published: October 2024

Abstract

Background
Impaired exercise capacity in adults with congenital heart disease is associated with mortality. Thus, exercise capacity is a potential tool for predicting risk in this population. The present study attempts to further explore the usefulness of exercise capacity in risk assessment of all-cause mortality, together with a limited number of easily accessible clinical variables.

Methods
The national register of congenital heart disease was searched for adults (18-60 years of age) with at least one bicycle ergometer exercise test and follow up in the register. The exercise capacity was classified based on percent of predicted exercise capacity (EC%pred); normal (&gt; 70%), moderately impaired (50-70%) or severely impaired (&lt;50%). All patients were followed for 10 years or until death, if occurring earlier than 10 years after the exercise test. Data were analyzed using logistic regression modelling. A risk evaluation model was constructed based on percent of predicted exercise capacity, age, and complexity of the heart lesion. Finally, the model was converted into a 5-point (p) risk score used to predict 10-year mortality (EC%pred &gt;70%=0p, 50-70=1p, &lt;50%=2p, age &lt;25=0p, 25-40=1p, 40-60=2p, simple lesion=0p, moderate/severe complexity of heart lesion=1p).

Results
1171 patients (mean age at exercise test 30.5 years, 44% women) were included with 70 deaths occurring within 10 years. A score 0-1 corresponded to &lt;1% 10-year all-cause mortality, 2p 5%, 3p 9%, 4p 21% and 5p 33% mortality risk (Table 1). The area under curve for the model was 0.75 (95% CI 0.69-0.81).

Conclusion
An easily performed risk assessment containing % of predicted exercise capacity, age and complexity of heart lesion performs well in detecting patients at both low and high risk of all-cause mortality within 10 years after an exercise test.