bluuebunny/crossref_metadata_2025_split huggingface dataset:
Efficacy of intravenous iron therapy compared to usual treatment in iron deficient adult cyanotic congenital heart disease patients for improvement in clinical outcomes at three months
Authors: Abdullah Z , Subhash Singh Y K , Gupta S K , Agstam S , Ramakrishnan S K 
Published: November 2023

Abstract

Background
Iron deficiency is frequent in CCHD patients and is associated with poorer outcomes. Patients with iron deficiency anaemia are treated with oral iron which results in improved clinical outcomes. The use of intravenous iron in CCHD patients is not well studied.

Purpose
We aimed to compare effectiveness of intravenous iron with usual care (i.e., no therapy for CCHD patients with iron deficiency without anemia, and oral iron therapy for CCHD patients with iron deficiency anemia) on 6-minute walk distance(6MWD) after 3 months of treatment, improvement in arterial Haemoglobin saturation, Quality of life, WHO functional class and safety of intravenous Iron.

Methods
We enrolled 27 patients of which 13 were randomized to IV iron arm and 14 to usual treatment arm. The mean age (±SD) of enrolled patient was 23.3 (±5.55) years. 24 out of 27 patients (88.9%) were Eisenmenger Syndrome patients, while the other three patients were TOF with pulmonary atresia, DORV with pulmonary atresia and post-Fontan. 6MWD was performed to access functional capacity and WHOQOL-BREF questionnaire was used for assessment of quality of life. All the clinical findings, data and test recordings were repeated after three months.

Results
The mean (±SD) haemoglobin(Hb) of patients included in the study was 16.7 (±2.0) g/dl. The Hb levels improved by 0.91 (±0.85) g/dl in patients who received any form of iron therapy (IV or oral iron). Hb improvement was not significantly different between those who received IV or oral iron [IV iron: 1.15 (±0.88) g/dl, Oral iron: 0.59 (±0.79) g/dl; p= 0.12]. The baseline 6MWD was not different in both the arms (p= 0.639). The mean (±SD) 6MW distance covered in IV iron arm and usual treatment arm was 401 (±35.2) m and 408.6 (±46.5) m respectively at baseline, which improved to 425 (±28.7) m and 421.5 (±43.2) m respectively at 3 months follow up. The change in 6MWD in IV iron arm and usual treatment arm was 24 (±22.3) m and 13.1 (±16.4) m respectively. The difference of change in 6MWD between the two arms was non-significant (p= 0.157). There was a significant improvement in WHOQOL-BREF Score Domains 2,3 and 4 and there was a trend towards improvement on Domain 1 score in IV iron arm compared to usual treatment arm. WHO functional class was similar at baseline in both arms (p= 0.534). The change in WHO functional class at follow up was similar in both arms (p= 0.082)

Conclusion
This study demonstrated no improvement in clinical outcomes of 6MW distance, arterial hemoglobin saturation and WHO functional class. However, there was significant improvement in quality of life and iv iron was found safe and equally efficacious as oral iron for correcting iron deficiency. Further studies for determining ideal hemoglobin levels and dose of iron in CCHD patients are pertinent.
Diabetic atrial cardiomyopathy can be identified by multiparametric mri MRI associating atrio-ventricular groove adipose tissue and left atrial strain profiles
Authors: Bialobroda J , Bouazizi K , Pouniah M , Kachenoura N , Zarai M , Charpentier E , Aron-Wisnewsky J , Hatem S N , Redheuil A 
Published: November 2023

Abstract

Background
Epicardial adipose tissue (EAT) is tightly associated with neighboring myocardium with free diffusion between the two tissues of fatty acid, peptides and adipokines. In specific circumstances EAT can favors myocardial remodeling and, for instance, contribute to the risk of atrial fibrillation (AF). Thereby, there is a growing interest for EAT as a biomarker of AF however, which is limited by difficulties of delineation of EAT vs. other paracardial adipose tissues. The atrio-ventricular groove adipose tissue (GEAT) has been proven to be pure epicardial adipose tissue.

Purpose
We tested the feasibility and value of measuring pure EAT of the atrio-ventricular groove (GEAT) using routine cardiovascular magnetic resonance imaging (CMR) in a cohort of patients with distinct metabolic disorders and studied its correlates to determine a CMR cardiometabolic score.

Methods
One hundred patients from the METACARDIS cohort (EU FP7) who had MRI, all in sinus rhythm and without overt clinical heart disease, were divided into 4 groups based on distinct metabolic profiles: metabolic syndrome (MSD, n=25), severe obesity (SO, BMI ≥ 35 kg/m2, n=18), type-2 diabetes (T2D, n=42) and healthy controls (n=15). Atrioventricular groove EAT volume was measured from cine SSFP MRI images in four-, three-, two-chamber and short-axis views; atrial EAT volume was measured from four- and two-chamber views. Native T1-mapping values of EAT at the anterior atrioventricular groove excluding the coronary vessels were measured from the MOLLI sequence (basal short-axis slice).

Results
CMR was performed in 100 patients from the MetaCardis cohort. GEAT volume measured from end-diastolic long axis views was obtained in all patients with a high correlation between GEAT and atrial EAT (r=0.95; P&lt;0.0001). GEAT volume was higher in the 3 groups of patients with metabolic disorders and highest in MSD. Together with age, body mass, fat distribution, GEAT volume allowed to distinguish obese, T2D and MSD from controls. T2D patients were uniquely characterized in CMR by decreased GEAT T1 relaxation and decreased peak longitudinal left atrial strain. We developed a CMR score by logistic regression and Random Forest machine-learning algorithm combining GEAT volume, T1 and peak LA strain that identified T2D patients with an AUC of 0.81 (Se:77%, Spe:80%; 95%-CI 0.72–0.91, p &lt;0.0001).

Conclusion
The epicardial adipose tissue of the AV groove measured by CMR can be used as the proxy of atrial EAT and, in combination with atrial strain and T1 relaxation, allows the early identification of the atrial cardiomyopathy associated with diabetes. There is diagnostic value of a multiparametric CMR approach for the detection of subclinical atrial cardiomyopathy and potentially to identify patients at risk of atrial fibrillation and stroke.
Pulmonary arterial hypertension associated to toxic-oil syndrome. A long story short
Authors: Cruz Utrilla A , Ponz De Miguel I , Jimenez Lopez-Guarch C , Velazquez-Martin M , Gallego Zazo N , Tenorio-Castano J A , Ochoa Parra N , Lopez-Alacid I , Hinojosa W , Arribas-Ynsaurriaga F , Escribano-Subias P 
Published: November 2023

Abstract

Introduction
Pulmonary arterial hypertension (PAH) is occasionally related to previous use of drugs or toxins. The toxic oil syndrome (TOS) is a model of drug-induced PAH caused by the prior use of denatured rapeseed oil. This rare condition caused an acute epidemic disease in Spain, including the development of pulmonary arterial hypertension, with a mortality rate of 82% from 1981 to 1987. Several cases have been discovered in the successive years until 2018. The clinical profile and outcome of those patients with PAH-TOS diagnosed since 1988 are unknown.

Methods
Historical cases of PAH-TOS since 1988 were collected. We analysed baseline characteristics, genetic testing, and the rates of lung transplantation or mortality in PAH-TOS, comparing these outcomes with incident idiopathic and heritable PAH cases (i/hPAH) from the Spanish Registry of Pulmonary Hypertension (REHAP).

Results
Thirty-nine patients with PAH-TOS between 1988 and 2018 were selected for this study. The median time between the toxic intake and the disease onset was 20.3 years (Interquartile range: 14.3 – 25.6). At least one patient per year was identified in that period, with a mean incidence of 1.63 cases/per year. Genetic testing was done in 21 patients, using a 37-gene Next Generation Sequencing panel, not finding any pathogenic or likely pathogenic variant. Importantly, in 3 out of 39 cases, the disease resolved (7.7%), while 4 cases needed lung transplantation. The 10-year mortality was 35.9%.
Cases of PAH-TOS were compared with 200 incident patients with i/hPAH collected from 2003 onwards. PAH-TOS patients were significantly younger when compared with i/hPAH patients (p&lt;0.0035). At baseline, patients with PAH-TOS had higher haemodynamically severity when compared with i/hPAH (mean pulmonary artery pressure of 65.0 mmHg Vs 53.5 mmHg, and pulmonary vascular resistance of 15.1 Vs 12.1 Wood Units, respectively; p=0.003, and p=0.02). In PAH-TOS the 5- and 10-year survival free of lung transplantation was 81.3%, and 52.7%, similar to the rates in the i/hPAH group (76.6%, and 59.2%, respectively; Log-rank test p=0.881. Figure).

Conclusions
The toxic oil syndrome is a unique model of drug-induced PAH, with new cases diagnosed several years after the toxic exposure. We did not find genetic variants in PAH-SOT patients. As described in other drug-induced cases of PAH, some patients experience a resolution of the disease over time. The very long-term survival of these patients is similar to that of patients with idiopathic or heritable pulmonary arterial hypertension.Figure
The effects of empagliflozin on right ventricular adaptation to pressure overload
Authors: Axelsen J S , Ringgaard S , Nielsen-Kudsk J E , De Man F S , Andersen A , Andersen S 
Published: November 2023

Abstract

Background
Right ventricular (RV) failure is the prime cause of death in patients with pulmonary arterial hypertension. Novel treatment strategies that protect the RV are needed. Empagliflozin, a member of the new anti-diabetic sodium-glucose co-transporter-2 inhibitors, shows a cardioprotective effect on the left ventricle in clinical and preclinical studies, but its direct cardiac effects on the RV remain elusive.

Purpose
To investigate the effects of empagliflozin on RV dysfunction induced by pulmonary trunk banding (PTB).

Methods
Male Wistar rat weanlings (116±10 g) were randomized to PTB or sham surgery. One week after surgery, PTB animals were randomized to empagliflozin 20 mg/kg/day mixed in the chow (300 mg empagliflozin/kg chow) (PTB-empa, n=10) or standard chow (PTB-control, n=10). Sham rats (Sham, n=6) received standard chow. After five weeks, RV function was evaluated by clinically relevant methods including echocardiography, cardiac MRI, and invasive pressure-volume measurements.

Results
PTB increased RV end-systolic pressures four-fold and caused RV hypertrophy with a 55 % increase in RV/(left ventricle + septum) weight ratio in PTB-control rats compared with sham. RV failure was evident by a decrease in cardiac output (PTB-control: 1.07±0.06 mL/s vs sham: 2.06±0.24 mL/s, p=0.0136). PTB-empa rats had a slight but non-significant weight reduction and a 33 % increase in water intake compared with PTB-control, yet no differences in hematocrit or blood glucose were observed. Treatment with empagliflozin decreased RV end-systolic pressures by 11 % (PTB-empa: 81±3 mmHg vs PTB-control: 91±2 mmHg, p=0.0091) without any effects on RV cardiac output. This decrease in RV end-systolic pressure in PTB-empa rats was complemented by less decreased minimum dp/dt and less increased maximum dp/dt compared with PTB-control without changes in the ventriculo-arterial coupling (Ees/Ea) (PTB-empa: 0.58±0.08 vs PTB-control: 0.67±0.14, p=0.8170). Load-independent measures of RV systolic and diastolic function were not affected in PTB-empa rats compared with PTB-control.

Conclusion
PTB caused RV failure in all rats subjected to the procedure. Empagliflozin treatment showed promising effects in pressure overload induced RV failure by reducing end-systolic pressure. Our study supports that treatment with empagliflozin has no toxic or adverse effects on the failing RV. Further studies are needed to explore empagliflozin’s potential beneficial role against RV failure.
The effect of sildenafil on maternal mortality in pulmonary arterial hypertension associated with congenital heart disease
Authors: Nahdi N S , Dinarti L K , Hartopo A B , Anggrahini D W 
Published: November 2023

Abstract

Background
Maternal mortality in pulmonary arterial hypertension (PAH) is very high, around 30–56%, so that patients with PAH and congenital heart disease (CHD) are advised to prevent pregnancy with class I-C recommendations. If the pregnancy continues, it must be handled by a multidisciplinary team with pulmonary hypertension (PH) experts, at the PH referral center. One of the PH-specific drug included in the FDA criteria B for pregnant women available in Indonesia is sildenafil. Sildenafil was reported to improve clinical symptoms and pregnancy outcomes in pregnant women with mild-moderate PAH. Sildenafil also had a tendency to reduce all-cause mortality although it was not statistically significant. Pulmonary hypertension specific drugs could reduce maternal mortality15.8% in subjects with PAH. However, research on sildenafil in maternal clinical outcomes is still very limited.

Purpose
This study aims to determine the effect of sildenafil on maternal mortality with PAH associated with CHD.

Methods
This study is an analytic observational study with a retrospective cohort method. Patient data were taken from the maternal registry, the Congenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry, and from the medical records of our general hospital who met the research criteria from August 2015 to December 2022. We assess the effect of sildenafil compared with controlled (without sildenafil) group. The clinical outcome of the study was maternal death during pregnancy and/or after delivery up to 42 days due to all causes (all causes mortality).

Results
Of 68 pregnant women who met the inclusion and exclusion criteria there were 32 subjects in the sildenafil group and 36 subjects in the control group. Maternal mortality was found to be higher (17.6%) in the group without sildenafil vs 9.7% in the sildenafil group. The majority causes of death were HP crisis (7 patients), 1 patient died from thromboembolism (HELLP syndrome), and 1 patient died from massive bleeding due to retained placenta. In the bivariate analysis, it was found that the NYHA functional class had a significant effect on maternal mortality with a p value &lt;0.05, but not significantly from multivariate analysis. In the bivariate and multivariate analysis, general anesthesia had a statistically significant effect on maternal mortality with p=0.018. Of the 9 pregnant women who died, 5 patients received general anesthesia. Of the five patients, NYHA III-IV was found in 4 subjects (4/5 = 80%) and the other patient was NYHA II but subject came in at 63% saturation. All of the subjects are Eisenmeinger syndrome.

Conclusion
Sildenafil can reduce maternal mortality by 45% although it is not statistically significant. Further studies with larger data are needed to confirm this result.Basic Characteristics of SubjectsRelationship of Sildenafil with Maternal
Mechanical thrombectomy in intermediate- and high-risk acute pulmonary embolism: haemodynamic outcome at 3 months
Authors: Lauder L , Perez-Navarro P , Goetzinger F , Ewen S , Al-Ghorani H , Haring B , Lepper P , Kulenthiran S , Boehm M , Link A , Scheller B , Mahfoud F 
Published: November 2023

Abstract

Background
Haemodynamic data months after mechanical thrombectomy for pulmonary embolism (PE) are scarce.

Aims
To assess the haemodynamic effects of mechanical thrombectomy in acute PE with right heart overload.

Methods
In this prospective, open-label study, patients with acute symptomatic, computed tomography-documented PE with signs of right heart overload underwent mechanical thrombectomy using the FlowTriever System. Right heart catheterization was performed immediately before and after thrombectomy and after three months. Transthoracic echocardiography was performed before thrombectomy, discharge, and at three months. This analysis was done after the first 20 patients completed their three months of follow-up.

Results
Twenty-nine patients (34% female) underwent mechanical thrombectomy, of which 20 completed three months follow-up with right heart catheterization. Most patients (17%) were at high or intermediate-high (76%) risk and had bilateral pulmonary embolisms (79%). Before thrombectomy, systolic pulmonary arterial pressure (sPAP) was severely elevated (mean 51.3±11.6 mmHg). Mean sPAP dropped by -15.0 mmHg (95% confidence interval [CI]: -18.9 to -11.0; p&lt;0.001) immediately following the procedure and continued to decrease from post-thrombectomy to three months (-6.4 mmHg, 95% CI: -10-0 to -2.9; p=0.002) (Figure). Right ventricle (RV)/left ventricle (LV) ratio immediately reduced within two days by -0.37 (95% CI: -0.47 to -0.27; p&lt;0.001). The proportion of patients with a TAPSE/sPAP ratio &lt;0.31 mm/mmHg decreased from 28% at baseline to 0% before discharge and at three months (p=0.007). There were no procedure-related major adverse events.

Conclusions
Mechanical thrombectomy for acute PE was safe and immediately reduced PAP and improved right heart function. The reduction in PAP was maintained at three months follow-up.Systolic pulmonary artery pressure
Long-term risk of pulmonary embolism and deep venous thrombosis after COVID-19: a population-based cohort study
Authors: Sjoland H , Lindgren M , Toska T , Hansson P O , Glise Sandblad K , Bjorck L , Lundberg C E , Adiels M , Rosengren A 
Published: November 2023

Abstract

Background
Venous thromboembolism (VTE) (pulmonary embolism (PE) or deep venous thrombosis (DVT)) is common during acute COVID-19. Long-term excess risk has not yet been established.

Objective
To study long-term VTE risk after COVID-19.

Methods
Swedish citizens aged 18-84 years, hospitalized and/or testing positive for COVID-19 between January 1, 2020, and September 11, 2021 (exposed), stratified by initial hospitalization, were compared to matched (1:5) non-exposed population-derived subjects without COVID-19. Outcomes were incident VTE, PE or DVT recorded at &lt;60, 60−&lt;180, and ≥180 days.

Results
Among exposed patients, 48,861 were hospitalized for COVID-19 (mean age 60.6 years) and 894,121 were without hospitalization (mean age 41.4 years) with 2,380 (4.9%) and 718 (0.08%) cases of VTE &lt;60 days, compared to 142 (0.06%) and 1,033 (0.02%) among non-exposed controls (244,242 and 4,468,411, respectively). Among patients hospitalized for COVID-19, adjusted hazard ratios (HRs) during 60−&lt;180 days were 6.05 (95% confidence interval (CI) 4.80─7.62) for PE and 3.97 (CI 2.96─5.33) for DVT, compared to non-exposed with corresponding estimates among COVID-19 without hospitalization 1.17 (CI 1.01─1.35) and 0.99 (CI 0.86─1.15), based on 475 and 2,311 VTE events, respectively. Long-term (≥180 days) HRs in patients hospitalized for COVID-19 were 2.01 (CI 1.51─2.68) for PE and 1.46 (CI 1.05─2.01) for DVT while non-hospitalized had similar risk to non-exposed, based on 467 and 2,030 VTE events, respectively.

Conclusions
Patients hospitalized for COVID-19 retained an elevated excess risk of VTE, mainly PE, after 180 days, while long-term risk of VTE in individuals with COVID-19 without hospitalization was similar to the non-exposed.
Development and validation of a clinical prediction model for the diagnostic management of acute pulmonary embolism: an individual participant data meta-analysis
Authors: Geersing G J , Takada T , Kraaijpoel N , Klok F A , Stals M , Freund Y , Le Gal G , Ghanima W , Huisman M V , Kline J , Righini M , Roy P M , Wells P S , Van Smeden M , Van Es N 
Published: November 2023

Abstract

Background/Introduction
Risk stratification is used for decisions about imaging in patients with clinically suspected acute pulmonary embolism (PE).

Purpose
To develop a clinical prediction model that provides an individualized, accurate probability estimate for acute pulmonary embolism (PE) based on readily available clinical items and D-dimer concentrations.

Methods
An individual participant data meta-analysis was performed based on sixteen studies with data from 28,305 adult patients with clinically suspected PE from various clinical settings. A multilevel logistic regression model was built and validated including ten a priori defined objective candidate predictors to predict PE at baseline or during follow-up of 30-90 days. Multiple imputation was used for missing data. Backward elimination was performed with a P-value &lt; 0.10. Discrimination (c-statistic with 95% CI and prediction intervals [PI]) and calibration (outcome:expected [O:E] ratio and calibration plot) were evaluated based on internal-external cross-validation. The accuracy of the model was compared to algorithms based on the Wells score and D-dimer testing.

Results
The final model included age (in years), sex, previous VTE, recent surgery/immobilization, hemoptysis, cancer, clinical signs of DVT, inpatient status, D-dimer (in μg/L), and an interaction term between age and D-dimer. The pooled c-statistic was 0.87 (95%-CI, 0.85-0.89; 95%-PI, 0.77-0.93) and overall calibration was very good (pooled O:E ratio, 0.99; 95% CI, 0.87-1.14; 95% PI, 0.55-1.79). In the lower range of probabilities, the model slightly overestimated VTE probability. Discrimination of the current model in the validation data sets was better than that of the Wells score combined with a D-dimer threshold based on age (c- statistic 0.73; 95%-CI, 0.70-0.75) or structured clinical pretest probability (c-statistic 0.79; 95%-CI, 0.76- 0.81).

Conclusion(s)
The present model provides an absolute, individualized probability of PE presence in a broad population of patients with suspected PE, with very good discrimination and calibration. Its clinical utility needs to be evaluated in a prospective management or impact study.Calibration plot of the new model