bluuebunny/crossref_metadata_2025_split huggingface dataset:
Evaluation of Perugini score and myocardial mass and voltage abnormalities in transthyretine cardiac amyloidosis
Authors: Costa J , Bichon L , Maouche A , Durdon P , Pouy R , Robbins A 
Published: October 2021

Abstract

Background
Transthyretine cardiac amyloidosis (TTR-CA) is an emerging treatable disease in cardiology. Severity of TTR infiltration can be assessed by bone scintigraphy with Perugini score, depending on HPD-Tc99M myocardial intensity uptake. Less is known on how Perugini score interplays with routine cardiac structural parameters, especially cardiac mass and voltage.

Aim
To evaluate correlation between Perugini score and myocardial mass and voltage abnormalities in patients with TTR-CA.

Methods
Patients diagnosed with ATTR-CA and addressed to our center for treatment underwent complete evaluation including electrocardiogram (ECG) and transthoracic echocardiography (TTE). Routine electrical and echographic parameters were obtained such as: Sokolow indice (SV1+RV5) in millivolt (mV), PR delay (ms), QRS width (in ms), left ventricular mass (LVM, in grams) and diastolic interventricular septum (dIVS) thickness (in mm). Patients were classified in three categories according to Perugini score: I (cardiac uptake &lt; chest bones), II (cardiac uptake = chest bones) and III (cardiac uptake &gt; bones). Main end-point were LVM and Voltage/Mass ratio (VMR) variations, depending on Perugini score.

Results
Among the 17 patients included, 14 were male (82.4%) and mean age was 82±8 years old. On ECG, 6 patients (35.3%) had permanent atrial fibrillation (AF). Mean voltage according to Sokolow index, mean PR delay and mean QRS width were respectively 1.5±0.5 mV, 212±54 ms and 113±19 ms. On TTE, mean LVM and mean dIVS width were respectively 333±98 g (188±55 g/m2) and 19±4 mm. Mean voltage/mass ratio (VMR) was 0.48±0.26 uV/g (see table). On bone scintigraphy, 3 patients were scored with Perugini I (17.6%), 4 with Perugini II (23.5%) and 10 with Perugini III (58.8%). According to Pearson test, there was a strong correlation between Perrugini score and LVM (r=+0.56, p=0.03) (graph A), and a strong negative correlation between Perrugini score and VMR (r=−0.70, p=0.003) (graph B). There were a trend for correlation between Perugini score and Voltage according to Sokolow (r=−0.46, p=0.07) and between Perugini score and dIVS width (r=0.49, p=0.07).

Conclusion
In TTR-CA patients, Perugini score appears to strongly correlate with left ventricle mass and voltage. Larger scale studies are needed to confirm these results.

Funding Acknowledgement
Type of funding sources: None. ECG and TEE trends according to PeruginiCardiac mass and voltage with Perugini
Cognitive disorders in takotsubo syndrome: incidence, short and long-term outcome. Results from a prospective multi-center registry
Authors: Mallardi A , Santoro F , Leopizzi A , Vitale E , Iacoviello M , Brunetti N D 
Published: October 2021

Abstract

Background
Takotsubo syndrome (TTS) is an acute and reversible left ventricular dysfunction, whose pathophysiological mechanisms are not completely known. There are evidence suggesting a possible link between neurological disease and TTS. Aim of the study was to evaluate incidence and prognostic value of cognitive neurological disorders among TTS patients.

Methods
379 consecutive patients were enrolled in a prospective multicenter registry. History, clinical features, echocardiographic parameters, in-hospital complications and long-term follow up events of all patients were recorded. Cognitive neurological disorders included Alzheimer disease, old age dementia and cognitive impairment for other causes.

Results
Prevalence of cognitive neurological disorders among TTS patients was 5.5% (num=21). Among this subset of patients 48% (num=10) had Alzheimer syndrome, 24% (num=5) old age dementia and 28% (num=6) cognitive impairment for other causes.
Compared to the control group, these patients were older (81±5 vs 71±12, p=0.01) and predominantly men (24% vs 9%, p=0.01). No differences in term of cardiovascular risk factors and left ventricular ejection fraction at admission and discharge were found among the two groups.
TTS patients with cognitive neurological disorders experienced higher rate of in-hospital complications (62% vs 28% p=0.01), that were mainly driven by higher rate of pulmonary edema (14% vs 9% p=0.01), cardiogenic shock (29% vs 8%, p=0.01), death (24% vs 4% p=0.01), ischemic stroke (10% vs 4% p=0.01) and left ventricular thrombi (10% vs 3%, p=0.01).
At long term follow up patients with cognitive neurological disorders when compared to those without, experienced higher rate of mayor cardiovascular events (48% vs 16%, p=0.01), cardiovascular re-hospitalization (14% vs 10%, p=0.01) and death (43% vs 9%, p=0.01).

Conclusion
TTS patients with cognitive neurological disorders had an increased risk of in and out of hospital mayor cardiac adverse events and mortality at short and long-term follow-up.

Funding Acknowledgement
Type of funding sources: Public hospital(s). Main funding source(s): Ospedali Riuniti di Foggia-Università di Foggia
Chronic kidney disease in patients with congenital heart disease – a nationwide cohort study
Authors: Gillesen M G , Fedchenko M F , Giang W G K , Dimopoulos K D , Eriksson P E , Dellborg M D , Mandalenakis Z 
Published: October 2021

Abstract

Introduction
Previous studies have shown an association between patients with congenital heart disease (CHD) and chronic kidney disease (CKD). We recently observed an increased prevalence of CKD in children and young adult CHD population, and as nowadays the majority of patients survives into adulthood this might become an increasing complication. However, it is not clear there is an increased risk to develop CKD in CHD patients compared to those without CHD.

Purpose
To investigate the incidence, risk and risk factors for CKD in children and adults with CHD (0–47 years old), compared to age- and sex-matched controls without CHD.

Method
Patients born with CHD between 1970 and 2017 were identified using data from the Swedish health register. Each patient with CHD was matched by birth year and sex to ten controls without CHD from the general population. Follow-up data were collected until 2017.

Results
We identified 71,936 CHD patients (52% male) who were matched to 714,457 controls during mean follow-up of 17.6 years (±12.8). Overall, 379 (0.5%) patients with CHD and 679 (0.1%) controls developed CKD; the risk of CKD was 6,4 times (95% confidence interval (CI): 5.65–7.27, p&lt;0.001) higher in patients with CHD compared to controls. The greatest risk of CKD was in patients with severe non-conotruncal defects (HR 11.31, 95% CI: 7.37–17.36, p&lt;0.001). The incidence of CKD increased exponentially with age but was in patients with and without CHD; however, at age of 47 years the cumulative incidence of CKD was 2.2% and 0.5% in patients with and without CHD respectively. Both absolute and relative risk of CKD was greater in CHD patients in the recent birth cohort (born between 1997–2017) (HR 9.98, 95% CI: 8.05–13.37) (incidence 28.1 per 100,000 person-years) compared to older patients (1970–1996) (HR 5.02 95% CI: 4.28–5.89) (incidence 39.5 per 100,000 person-years). Diabetes mellitus was the only risk factor that associated with the risk of CKD in CHD patients.

Conclusion
In the present nationwide cohort study, we found that patients with CHD have an over 6-fold risk of developing CKD compared to matched controls without CHD. The incidence of CKD increases with age, but also younger CHD patients are at risk, prompting action to establish regular follow-up of renal function in all patients with CHD, and identify individuals at risk as early as possible. Further mapping of risk factors and effects of kidney disease on the CHD population is also required.

Funding Acknowledgement
Type of funding sources: Public hospital(s). Main funding source(s): The Swedish state and the Swedish Research Council
Infective endocarditis in adult patients with congenital heart disease: results from the ESC EORP EURO-ENDO registry
Authors: Van Melle J , Roos-Hesselink J , Bansal M , Kamp O , Meshaal M , Pudich J , Reskovic Luksic V , Rodriguez-Alvarez R , Sadeghpour A , Separovic Hanzevacki J , Sow R , Timoteo A T , Laroche C , Lancellotti P , Habib G ,   EURO-ENDO Investigators group
Published: October 2021

Abstract

Background
Congenital Heart Disease (CHD) predisposes to Infective Endocarditis (IE)

Purpose
To characterize and to determine the prognosis of IE in this specific population.

Methods
The ESC EORP EURO-ENDO study is a prospective international study in patients (n=3111) diagnosed with IE. In this pre-specified ancillary analysis, we aimed to describe adult patients with CHD (n=365, 11·7%) and compare them with patients without CHD (non-CHD, n=2746) in terms of baseline characteristics and 1-year outcome.

Results
CHD patients (73% men, age 44·8±16·6 years) were younger and had less comorbidities. Of the CHD patients, 14% had a dental procedure in the 6 months before hospitalization versus 7% in non-CHD patients (p&lt;0·001) and more often positive blood cultures for Streptococcus viridans (16·4% vs 8·8%, p&lt;0·001). As in non-CHD patients, IE affected most often the left-sided valves. For CHD patients, in-hospital mortality was 9·0% vs 18·1% in non-CHD patients (p&lt;0·001), and also 1-year outcome was more favourable (log-rank for all-cause mortality p&lt;0·0001), even after adjustment for age (Hazard Ratio (HR) 0·61; 95% CI 0·45–0·81). Within the CHD population, multivariable Cox regression revealed the following predictors for mortality: fistula (HR 6·97), cerebral embolus (HR 4·64), renal insufficiency (HR 3·44), Staphylococcus aureus as causative agent (HR 2·06) and failure to undertake surgery when indicated (HR 5·93).

Conclusion
CHD patients with IE have better outcome in terms of all-cause mortality. The observed high incidence of dental procedures prior to IE fuels the discussion about the need for antibiotic prophylaxis.

Funding Acknowledgement
Type of funding sources: Other. Main funding source(s): Since the start of EORP, the following companies have supported the program: Abbott Vascular Int. (2011–2021), Amgen Cardiovascular (2009–2018), AstraZeneca (2014–2021), Bayer AG (2009–2018), Boehringer Ingelheim (2009–2019), Boston Scientific (2009–2012), The Bristol Myers Squibb and Pfizer Alliance (2011–2019), Daiichi Sankyo Europe GmbH (2011–2020), The Alliance Daiichi Sankyo Europe GmbH and Eli Lilly and Company (2014–2017), Edwards (2016–2019), Gedeon Richter Plc. (2014–2016), Menarini Int. Op. (2009–2012), MSD-Merck & Co. (2011–2014), Novartis Pharma AG (2014–2020), ResMed (2014–2016), Sanofi (2009–2011), Servier (2009–2021), Vifor (2019–2022). Survival CHD vs non-CHD patients
A comparison of ECG-based home monitoring devices in adults with congenital heart disease
Authors: Bokma J P , Pengel L K D , Robbers-Visser D , Groenink M , Boekholdt S M , Bouma B J 
Published: October 2021

Abstract

Background
Various devices are available for electrocardiogram (ECG) based home monitoring and could facilitate early detection of arrhythmias in adult congenital heart disease (ACHD). However, the diagnostic accuracy of these devices in ACHD is unknown.

Purpose
We aimed to determine the accuracy of different ECG-based devices compared to the standard 12-lead ECG in ACHD.

Methods
We performed a single-center, prospective, cross-sectional study in consecutive ACHD patients. We determined diagnostic accuracy of the Withings Scanwatch (lead I), Eko DUO (precordial ECG) and Kardia 6L (six leads) compared to the 12-lead ECG on several parameters: 1) automatic atrial fibrillation (AF) detection, 2) QRS-morphology assessment (simplified into normal (&lt;120ms), right bundle branch block (&gt;120ms) or other wide complexes (&gt;120ms)), and 3) conduction times (PR, QRS, QTc time). All 12-lead and device ECGs were evaluated by a single observer.

Results
A total of 176 patients (54% male, age 40±16.6 years, 24% severe ACHD, 84% previous correction, six patients with AF) enrolled in the study. The Withings algorithm classified only 5% of ECGs as inconclusive compared to 31% of Kardia 6L (p&lt;0.001). Both devices had comparable sensitivity and specificity (Withings: 100% and 100% vs. Kardia 6L: 100% and 97%) in ECGs that were classified by the algorithm. On Kardia 6L, QRS morphology was correctly classified more frequently (90%) compared to EKO duo (84%) (p=0.03) and identified the P-wave more frequently (85%) (p&lt;0.001 compared to both). Evaluation of all ECG-based devices underestimated QTc and QRS duration compared to the 12L ECG (p&lt;0.01). Withings (49%) more frequently over- or underestimated QTc-interval by more than 40msec compared to Eko DUO (30%) and Kardia 6L (26%) (p&lt;0.001 for both). Overall, ECG quality of Withings Scanwatch was rated worse compared to Kardia 6L and EKO duo (p&lt;0.001 for both).

Conclusions
This is the first study to examine diagnostic accuracy of different ECG-based devices in ACHD. Overall, Withings Scanwatch may be the most practical choice for continuous AF screening while Kardia 6L could be preferred to accurately capture symptomatic palpitations. These findings can inform both patients and caregivers for optimal choice of home monitoring.

Funding Acknowledgement
Type of funding sources: None.
Hemodynamic effects of balloon pulmonary angioplasty for the treatment of total and subtotal pulmonary artery occlusions in inoperable chronic thromboembolic pulmonary hypertension
Authors: Stepniewski J , Magon W , Przybylski R , Kopec G 
Published: October 2021

Abstract

Background
Balloon pulmonary angioplasty (BPA) has become a therapeutic option for inoperable chronic thromboembolic pulmonary hypertension (CTEH). Despite significant improvement in the technique, treatment of subtotal (STO) and total (TO) pulmonary artery occlusions with BPA may pose risk, but the efficacy is unknown.

Aim
We aimed to study to assess safety and efficacy of BPA in STO/TO.

Methods
We included consecutive patients with inoperable CTEPH, who underwent BPA treatment. To evaluate the efficacy and safety we grouped all BPA sessions into those in which recanalization of at least one STO or TO was performed and into those without. The primary efficacy outcome was improvement in pulmonary vascular resistance (PVR) in BPA sessions with STO/TO recanalization as compared to those without.

Results
We analyzed 169 BPA sessions in 50 CTEPH patients. Out of a total number of 832 lesions subjected for BPA, 168 were classified as STOs or TOs [129 (15,5%) and 39 (4,7%) respectively]. Three (2,3%) STOs and 8 (20,5%) TOs were not recanalized despite repeated attempts. There were 90 BPA sessions with at least one STO/TO recanalization. Racanalization of at least one STO/TO was associated with a trend towards PVR improvement as compared to non-STO/TO BPAs (−69±162 vs −38±135 dyn s cm–5, p=0,19). Recanalization of STO/TO at the level of segmental pulmonary artery as compared to subsegmental-only STO/TO recanalizations or no-STO/TO recanalization was associated with significant PVR improvement (−126±192 vs −38±135 dyn s cm–5, p=0.007). The rate of complications was similar in STO/TO and non-STO/TO BPA sessions (14.4% vs 12.6%, p=0.56).

Conclusions
The use of BPA for the recanalization of subtotal and total PA occlusions is safe and effective. Recanalization of segmental STO/TOs leads to significant improvement in PVR.

Funding Acknowledgement
Type of funding sources: None.
Efficacy and safety of early switch to oral anticoagulation in acute intermediate-risk pulmonary embolism
Authors: Klok F A , Toenges G , Barco S , Konstantinides S V ,   PEITHO-2 investigators
Published: October 2021

Abstract

Background
Guidelines recommend risk-adjusted management of pulmonary embolism (PE). We investigated whether treatment of acute intermediate-risk PE with parenteral anticoagulation for a short period of 72 hours, followed by switch to a direct oral anticoagulant (dabigatran), is effective and safe.

Methods
We conducted a prospective multicentre single-arm management trial in patients with intermediate-risk PE (EudraCT Identifier 2015–001830–12). Patients received parenteral heparin, switched to dabigatran 72 hours after diagnosis following standardised clinical assessment. The study was terminated early following sample size adaptation after the predefined interim analysis.

Findings
From January 2016 through July 2019, 402 patients were enrolled in 9 European countries. Adherence to protocol was 92.0% (Table 1). The primary outcome, recurrent symptomatic venous thromboembolism or PE-related death within 6 months (Table 2), occurred in 7 patients (1.7%; upper bound of right-sided 95% CI 3.2%; p=0.ehab724.192322 for rejecting H0), with all events in the intermediate-high-risk group (2.5%; upper bound of right-sided 95% CI 4.6%). Median duration of hospitalisation for the index event was 6 days, despite the fact that &gt;70% of the patients had intermediate-high-risk PE. The six-month incidence of major bleeding was 2.7%; the only fatal haemorrhage occurred before switch to dabigatran.

Interpretation
A strategy of early switch from heparin to dabigatran following standardised clinical assessment was effective and safe in intermediate-risk PE. Our results can help to fine-tune guideline recommendations for initial treatment of PE, optimising the use of resources and avoiding prolonged hospitalisation.

Funding Acknowledgement
Type of funding sources: Public grant(s) – National budget only. Main funding source(s): German Federal Ministry of Education and Research; and Boehringer Ingelheim Table 1. Time between diagnosis of pulmonary embolism and switch to dabigatran (N = 402 patients)Table 2. Efficacy and safety outcomes
The Birmingham Black Country Venous Thromboembolism (BBC-VTE) registry of outcomes of venous thromboembolism patients admitted to hospital
Authors: Sanders A , El-Bouri W K , Lip G Y H ,   Birmingham Black Country Venous Thromboembolism Investigators
Published: October 2021

Abstract

Background
Venous thromboembolism (VTE) is a condition which causes significant morbidity and mortality in hospitalised patients as well as in the community. These are related to adverse outcomes associated with the first incidence of VTE, such as recurrence, major haemorrhage and mortality. These outcomes are difficult to measure and compare among the different regions of the world not least because of varying prevalence of risk factors, paucity of comparable studies and a differing approach to treatment of VTE (1–3).

Purpose
Our VTE registry aims to estimate the above mentioned outcomes as applicable to a developed world population with a multi-ethnic background. Furthermore, our registry has provided a data set to develop and validate a machine learning algorithm in order to predict outcomes in patients admitted to hospital with VTE. This will have profound implications for clinicians who will be able to make evidence-based decisions about which patients are low risk and so can be discharged early as well as those who are high risk and may need more intensive follow up.

Methods
BBC-VTE registry is a retrospective, multi-centre, observational registry. We identified all patients (N=1554) who were admitted with a final radiologically confirmed diagnosis of pulmonary embolism and/or lower limb deep vein thrombosis at three regional hospitals in the UK during the years 2012–2014. Each patient's electronic record was accessed by clinicians to confirm radiological diagnosis of VTE and also collect data on demographics, physical examination findings and laboratory analysis on admission, past medical history, and treatment plan. Outcomes were also recorded including recurrence of VTE, subsequent major bleeding and all-cause mortality.
A simple multivariate analysis (logistic regression) was used to determine risk factors associated with all-cause mortality. Odds ratios (OR) and 95% CI are reported.

Results
The main factors determining higher all-cause mortality were age, a history of diabetes, admission laboratory analysis (c-reactive protein and neutrophil count), and previous malignancy (OR &gt;1) (see Fig. 1). Conversely, hypercholesterolaemia, discharge oral anticoagulation, immobilization, and post-PE syndrome were all significantly correlated with a reduced risk of mortality (OR &lt;1).

Conclusions
BBC-VTE provides unique data on VTE mortality risks in a multi-ethnic cohort. The strengths of our registry are that we are only including radiologically verified VTE patients unlike many of the epidemiological studies to date which rely on clinical coding. We have also shown that it is possible to risk-stratify patients admitted with VTE using simple clinical variables which has implications for their discharge decisions.

Funding Acknowledgement
Type of funding sources: None. Figure 1
Impact of left heart disease risk factors on risk stratification and treatment response in pulmonary arterial hypertension
Authors: Kearney K , Anderson J , Cordina R , Lavender M , Celermajer D , Collins N , Dwyer N , Keating D , Williams T , Whitford H , Whyte K , Weintraub R , Keogh A , Kotlyar E , Lau E M T 
Published: October 2021

Abstract

Background
Contemporary registries have documented a change in the epidemiology of PAH patients displaying increasing co-morbidities associated with left heart disease (LHD). These patients are often excluded from randomized clinical trials. It is unclear whether the presence of LHD comorbidities may adversely impact the accuracy of risk stratification and response to PAH therapy.

Method
Data was extracted from the Pulmonary Hypertension Society of Australia and New Zealand registry for incident patients with a diagnosis with idiopathic/heritable/toxin-induced (I/H/D)-PAH and connective tissue disease (CTD) associated PAH from 2011 - 2020. Patients without available medication and follow up data were excluded. We used the AMBITION trial exclusion criteria to define the subpopulation with LHD risk factors and haemodynamic phenotype (PAH-rLHD).

Results
489 patients (I/H/D-PAH=251, CTD-PAH=238) were included in our analysis, with 103 (21.0%) fulfilling the definition of PAH-rLHD (34 had ≥3 risk factors for left heart disease (rLHD-hypertension, diabetes, obesity or ischaemic heart disease) and 76 had borderline haemodynamics (mean capillary wedge pressure 13–15 with pulmonary vascular resistance &lt;500 dynes sec/cm5) including 7 who met both criteria). Compared to classical PAH, patients with PAH-rLHD were older at diagnosis (66±13 vs 58±19, p&lt;0.001), had lower pulmonary vascular resistance (PVR: 393±266 vs 708±391, p=0.031) but worse exercise capacity (6MWD: 286±130m vs 327±136m, p=0.005). PAH-rLHD were more likely to be started on initial monotherapy, compared with “classical” PAH (73% vs 56%, p=0.002). In the monotherapy groups, endothelin receptor antagonists (ERA) were used in 73% PAH-rLHD, compared with 66% in classical PAH group. Both groups exhibited similar response to both mono- and combination therapy with commensurate improvements in WHO functional class (mean change 0.3±0.6 vs 0.3±0.8, p=0.443) and 6-minute walk distance (mean change 44±82 vs 48±101, p=0.723). There was no difference in survival between classical PAH and PAH-rLHD (log rank, p=0.29). The REVEAL 2.0 risk score effectively discriminated risk in both populations at baseline and first follow up (classical PAH: baseline C statistic 0.750, follow up 0.774 and PAH-rLHD: baseline C statistic 0.756, follow up 0.791).

Conclusion
Despite lower PVR at diagnosis, PAH-rLHD patients and “classical” PAH demonstrate similar response to first-line therapy with similar long term survival. The REVEAL 2.0 risk score can be effectively applied to the subpopulation of PAH-rLHD in real life clinical practice.

Funding Acknowledgement
Type of funding sources: None.