bluuebunny/crossref_metadata_2025_split huggingface dataset:
The patient with Down syndrome and pulmonary arterial hypertension associated with congenital heart disease: from childhood to adulthood
Authors: Playan Escribano J , Garrido-Lestache E , Luna R , Alvarez Fuente M , Lopez-Meseguer M , Guillen Rodriguez I , Perez Penate G M , Sabate Rotes A , Elias Hernandez T , Lopez Ramon M , Rueda Soriano J , Perin F , Blanco I , Del Cerro Marin M J , Escribano Subias P 
Published: October 2022

Abstract

Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD) that significantly affects its prognosis. Down syndrome is a common comorbidity among patients with PAH associated with CHD. Children born in recent years with CHD have benefited from advances in pediatric cardiac surgery that have modified their evolution compared to adults with the same pathologies. Our objective was to know and compare the characteristics of the adult and pediatric population with DS and PAH associated with CHD.
REHAP and REHIPED are Spanish, voluntary, multicenter registries that include patients with PAH associated with CHD. REHAP started in 2007 and includes patients over 18 years of age. Patients diagnosed with PAH after this time were prospectively included, until 2021. Patients who were followed in the participating centers and diagnosed after January 1998 were retrospectively included in the registry. REHIPED started in 2009 and collects patients between 2 months and 18 years of age. Patients diagnosed with PAH after this time were prospectively included, until 2021. Patients who were followed and diagnosed after January 1998 were retrospectively included. 131 adults and 47 children with DS and PAH associated with CHD were analyzed. Their baseline characteristics, hemodynamics, treatment, and 10-year survival were compared.
Adult patients with DS presented more frequently complete atrioventricular canal defect as underlying heart disease and were mostly in Eisenmenger's situation (84%). In pediatric patients the largest group was post-repair PAH (50%). Functional class was similar in both groups, although adults presented significantly greater hemodynamic severity. Regarding medical treatment, no differences were observed between both groups: there was a very low use of prostanoids both at baseline and at follow-up, and a majority use of oral monotherapy. There were no significant differences in survival.
Corrective surgery in patients with DS has become more frequent over the years: adults are mostly in Eisenmenger's situation, while post-repair PAH is the most numerous group in children. Although adults show greater hemodynamic severity, survival is similar in both groups. Medical treatment, which showed no differences between pediatric and adult age, is very conservative, with most patients on oral monotherapy and rare use of prostanoids.

Funding Acknowledgement
Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Janssen MSD
Catheter ablation for atrial fibrillation in adult congenital heart disease: an international registry study
Authors: Griffiths J , Liang J , Khairy P , Srivatsa U N , Frankel D , Sandhu A , Shoemaker M B , Natale A , Lakkireddy D , De Groot N M S , Gerstenfeld E , Moore J P , Avila P , Ernst S , Nguyen D T 
Published: October 2022

Abstract

Background
Life expectancies for patients with congenital heart disease (CHD) have dramatically increased in recent years, accompanied by a rise in atrial fibrillation (AF) prevalence. Data on AF ablation strategy and outcomes are limited in CHD.

Purpose
We aimed to investigate the characteristics of CHD patients presenting for AF ablation and their outcomes.

Methods
A multicenter, retrospective analysis was performed of CHD patients undergoing AF ablation between 2004 and 2020 at 13 participating centers. The severity of CHD was classified using the 2014 PACES/HRS guidelines. Clinical data were collected including ablation strategy and follow up. One-year procedural success was defined as freedom from AF in the absence of antiarrhythmic drugs (AADs, complete) or including previously failed AADs (partial).

Results
Of 240 patients, 127 (53.4%) had persistent AF, 62.5% were male, and mean age was 55.2±0.9 years. CHD complexity categories included 147 (61.3%) simple, 69 (28.8%) intermediate and 25 (10.4%) severe. The most common CHD type was atrial septal defect (n=78). More complex CHD conditions included transposition of the great arteries (n=14), anomalous pulmonary veins (n=13), tetralogy of Fallot (n=8), cor triatriatum (n=7), single ventricle physiology (n=2), among others. The majority (71.3%) of patients had AF despite at least one AAD. 46 patients (22.1%) had a reduced systemic ventricular ejection fraction &lt;50%, and the mean left atrial diameter was 44.1±0.7 mm. PV isolation (PVI) was performed in 227 patients (94.6%); additional ablation strategies included left atrial linear ablations (25.4%), CFAE (19.2%), and cavotricuspid isthmus ablation (40.8). One-year complete and partial success rates were 45.0% and 20.5%, respectively, with no significant difference in the rate of complete success between complexity groups. Overall, 38 patients (15.8%) required more than one ablation procedure. There were 3 (1.3%) major and 13 (5.4%) minor procedural complications.

Conclusion
AF ablation in this complex population was safe and resulted in AF control in the majority of patients. Future work should address the most appropriate ablation targets in the challenging population.

Funding Acknowledgement
Type of funding sources: None.
Preferences for disease-related information and transitional skills among adolescents with congenital heart disease in the early transitional stage
Authors: De Hosson M , Goossens E , De Groote K , Muino Mosquera L , Wynendaele H , De Backer J 
Published: October 2022

Abstract

Introduction and aims
The transition towards adult-focused healthcare comprises a complex process through which adolescents with congenital heart disease (CHD) should be carefully guided. Various task forces encourage implementing person-tailored transition programs, but detailed data on adolescents' preferences regarding disease-related information and acquirable transitional skills are mostly lacking. This study examined these preferences towards adolescents with moderately and severely complex CHD.

Material and methods
A cross-sectional descriptive study including logistic regression analysis was performed in a Belgian tertiary CHD center with adolescents recruited from “Transition With a Heart” (TWAH). For this 4-year transition program, adolescents were invited to complete a communication paper (focused on the need for information) and an individualized transition plan (focused on the evaluation of transitional skills) prior to an outpatient visit. Only questionnaires completed in the early transition stage, from January 2016 to August 2021, were used for data analysis.

Results
Forty-nine adolescents completed both questionnaires at an average age of 15.9±1.2 years. Of all adolescents, 59% indicated the need for information about the heart defect and surgical treatment, followed by 45% that expressed the need for information about sports and medication; and ≤27% regarding psychosocially-related topics. The proportion of girls needing information was larger than boys. Significantly more girls than boys requested information about surgeries (76% versus 46%; p=0.04). Younger adolescents were more likely to express a need for information about most topics and significantly more likely regarding surgeries (odds ratio=0.53; 95% CI [0.26 to 0.88]; p=0.03).
A maximum of 14% of adolescents were interested in acquiring transitional skills. Larger proportions of adolescents perceived the relevant transitional skills as insufficient, from 21% to 83%.

Conclusion
The preferences for disease-related information of adolescents with CHD are mainly related to medical topics and tend to decrease with increasing age. This motivates timely gauging of individual information needs and delivering disease-related information, ideally in the early teens. Furthermore, preferences varied according to gender, with more information requested by girls.
Adolescents expressed minimal needs to acquire transitional skills despite the low self-perceived skills levels. This paradox requires further research to enable the adaptation of transition interventions.

Funding Acknowledgement
Type of funding sources: None.
The crucial role of tricuspid regurgitation in patients with pulmonary arterial hypertension
Authors: Moschella M M , Ghio S , Magrini G , Cannata A , Lo Giudice F 
Published: October 2022

Abstract

Introduction
Pulmonary arterial hypertension (PAH) is a rare and progressive disease with high morbidity and mortality rate and its prognosis is critically affected by right ventricle (RV) dysfunction. Several studies have demonstrated that the presence of moderate-severe tricuspid regurgitation (TR) is associated with poor prognosis in PAH. However, the echocardiographic correlates and the impact on functional capacity of significant TR in PAH patients have not been thoroughly investigated.

Purpose
The aims of this study were to confirm the prognostic role of moderate-to-severe TR in PAH patients and to assess the effects of significant TR on remodelling of right cardiac chambers evaluated by echocardiography and on exercise capacity determined by six minute-walking-test (6MWT) and cardiopulmonary exercise testing (CPET).

Methods
This study is a retrospective longitudinal analysis of 103 patients with newly diagnosed PAH enrolled from 2010 to march 2021. Patients with portopulmonary PAH and with congenital heart disease PAH were excluded. Median follow up (FU) was 27 months and the primary endpoint was all-cause mortality.

Results
Our cohort was characterised by high BNP mean values (382 pg/mL), poor exercise capacity (260 mt at 6MWT, peak VO2 11 mL/kg/min at CPET), right ventricular dysfunction and severe PH at right heart catheterization (cardiac index 2.3 L/min/mq, mean pulmonary artery pressure 48 mmHg, pulmonary vascular resistance 12 WU). Sixty-nine percent (70/103) of the cohort had significant TR. Thirty percent of patients (30/103) died during follow-up.
At Cox univariate regression, moderate to severe TR was significantly associated with higher risk of death (HR 4.48, p=0.004) (Figure 1). At bivariate analysis combining TR grade and TAPSE/sPAP, significant TR showed to be associated with higher risk of death (HR 3.59, p=0.05). In particular, the combination of normal TAPSE and mild TR identified a subgroup of patients who had no events during the follow-up period (Figure 2).
At echocardiography patients with moderate-to-severe TR had more peripheral venous congestion, worse right ventricular function (TAPSE and TAPSE/sPAP), lower stroke volume index. The presence of moderate-to-severe TR promoted negative remodelling of the right atrium and of the right ventricle, which was more spherical, hypertrophic and stiff. Exercise capacity of patient with significant TR was significantly reduced both at CPET and at 6MWT.

Conclusions
Moderate-to-severe tricuspid regurgitation is associated with poor exercise capacity (both at CPET and at 6MWT) and poor survival in patients with PAH. Echocardiography can easily detect morphological and functional abnormalities associated with a worse prognosis in patients with PAH and represents an important non-invasive tool for risk stratification.

Funding Acknowledgement
Type of funding sources: None.
The timing of death in acute pulmonary embolism patients regarding the mortality risk stratification at admission to hospital
Authors: Obradovic S , Dzudovic B , Matijasevic J , Salinger S , Kos L J , Benic M , Mitevska I , Kafedzic S , Kovacevic-Preradovic T , Neskovic A , Bozovic B , Bulatovic N , Miloradovic V 
Published: October 2022

Abstract

Background
The management of patients with acute pulmonary embolism (aPE) depend on the risk stratification at hospital admission. It is unknown when normotensive aPE patients with some other risk factors deteriorate.

Patients and methods
Patients with objectively established acute PE diagnosis enrolled in the regional PE registry from January 2015 to December 2021, were studied in this investigation. According to European Society od Cardiology criteria patients were stratified during admission to hospital in four risk stratums. The timing for death and the main reason for death were recorded. PE-related death was defined if patient has died because of cardiac arrest or obstructive shock if there is no another possible reason for that.

Results
In the REPER registry. Among 1541 patients (514 low risk, 366 intermediate-low risk, 472 intermediate-high risk and 189 high risk) with aPE, 101 (6.6%) have died primary from aPE and 64 (4.2%) have died from other reasons during the 30-day follow-up. PE-related death across the mortality risk groups were 0.8%, 1.1%, 8.5% and 28.5% in low-risk, intermediate-low, intermediate-high and high risk PE, respectively. Median time from hospital admission to PE related death was significantly longer in intermediate-high than in high risk patients 4.5 (2.0–9.0) vs 1.0 (1.0–4.5) days, p=0.001. In the high risk group 50.9% of patients died during the first 24 hours, 9.0% in the next 24 hours and 83.0% of patients died during the first 5 days from admission. In the intermediate-high risk group 17.5% died in the first 24 hours, 12.5% died in the next 24 hours and next 25% died till the fifth day. There was no difference in timing of non PE-related death between intermediate-high and high risk patients 9.5 (6.0–18.5) vs 7.0 (3.0–23.5) days, p=0.631.

Conclusion
There is significant delay in timing of death in intermediate-high compare to high risk PE patients, however, almost 50% of patients who died in the intermediate-high risk PE patients have died inside the first 5 days from hospital admission.

Funding Acknowledgement
Type of funding sources: None.
An updated meta-analysis of hemodynamics markers of prognosis in patients with pulmonary hypertension due to left heart disease
Authors: Caravita S , Baratto C , Soranna D , Dewachter C , Bondue A , Zambon A , Badano L P , Parati G 
Published: October 2022

Abstract

Background
Pulmonary hypertension (PH) is associated with a poor prognosis in patients with left heart disease (LHD). Several hemodynamic variables have been shown to predict outcome, including pulmonary vascular resistance (PVR), pulmonary artery compliance (PAC), and the diastolic pressure gradient (DPG). We sought to provide an updated analysis on the association of these variables with prognosis in PH-LHD.

Methods
We performed a systematic literature review including studies reporting association measurements between DPG and/or PVR and/or PAC and death in PH-LHD patients. These hemodynamic variables were extracted to estimate the pooled hazard ratio (HR) of adverse outcome for each one, and cumulative meta-analysis was performed to investigate temporal trends in the effects reported in the literature as well as the impact of sample size.

Results
17 articles were identified, including 9716 patients with LHD, heterogeneous in terms of age, sex, and etiology of cardiac disease. In this large population, we found that PVR (HR, 1.09; 95% CI: 1.06–1.12), DPG (HR, 1.02; 95% CI: 1.01–1.02) and PAC (HR, 0.73; 95% CI: 0.76–0.81) were associated with an increased risk of adverse outcome, albeit with a less solid performance of DPG (Figure 1). Similar results were found when hemodynamic variables were analyzed according to the thresholds commonly applied in clinical practice, or subdividing cohorts according to the underlying LHD (either heart failure with preserved or reduced left ventricular ejection fraction, or valvular heart disease). Furthermore, cumulative metanalysis indicated that these results are consistently stable since 2018 (Figure 2).

Conclusions
Despite the heterogeneity of PH-LHD group and the intrinsic limitations of each variable, PVR, DPG, and PAC have an established prognostic value in PH-LHD. The strongest correlation with PVR and PAC supports their use in defining disease severity and identifying a subgroup of patients at higher risk of adverse outcome. We believe that these results are consistent through the years and unlikely to change with the addition of further studies.

Funding Acknowledgement
Type of funding sources: Private grant(s) and/or Sponsorship.
Speckle-tracking global longitudinal strain predicts clinical outcomes in patients with systemic sclerosis
Authors: Stronati G , Paolini F , Benfaremo D , Selimi A , Dello Russo A , Moroncini G , Guerra F 
Published: October 2022

Abstract

Introduction
Systemic-sclerosis related cardiomyopathy is frequent and, albeit often asymptomatic, is likely secondary to the underlying pathophysiology of SSc itself and represents a negative prognostic factor. Speckle-tracking derived global longitudinal strain (GLS) has been proven to be a cost-effective tool in the detection of LV and RV dysfunction in patients with SSc and no overt cardiac disease.

Purpose
The aim of our study was to assess whether baseline GLS could predict clinical outcomes in patients with SSc in terms of death, heart failure, hospitalizations, and development of pulmonary hypertension.

Methods
We conducted a prospective observational study on all consecutive patients referred to our clinic between June 2016 and January 2022 with a confirmed diagnosis of SSc and no overt cardiac disease, pulmonary hypertension, or atrial fibrillation at the time of enrollment.
We performed baseline echocardiogram and GLS calculations for all patients as well as collected clinical and ECG data at baseline and at each follow up.

Results
Out of 164 patients (148 female, 58±14 years), 19 (11.6%) patients died during a median follow-up of 3.2 years for mainly non-cardiovascular deaths (7.3%) while cardiovascular deaths were lower (3% non-sudden, 1.3% sudden).
Left GLS at first visit was associated with all-cause death, with a 1% left GLS worsening associated with a 19% increased risk of death after adjusting for age, gender, and LVEF (adjusted HR 1.19; 95% CI 1.05–1.35; p=0.007).
Similarly, right GLS at first visit was associated with all-cause death, with a 1% right GLS worsening associated with a 12% increased risk of death after adjusting for age, gender, and TAPSE (adjusted HR 1.12; 95% CI 1.03–1.21; p=0.005).
Patients with a left GLS worse (i.e. higher) than −20% had a 3.5-fold increased risk of death when compared to patients with better left GLS (HR 3.55; 95% CI 1.28–9.88; p=0.015; Figure 1a). Similarly, patients with a right GLS worse than −20% had a 4.5-fold increased risk of death when compared to patients with better left GLS (HR 4.47; 95% CI 1.4–13.74; p=0.009; Figure 1b).

Conclusions
Baseline GLS measured is an effective tool to recognize patients with SSc and negative prognosis. It may help us identify early those who need to be followed more closely and start appropriate treatment in order to prevent major cardiovascular and non-cardiovascular events. The close relationship between left and right GLS as risk predictors underlines the role of SSc in promoting overt cardiac disease, not limited to pulmonary hypertension.

Funding Acknowledgement
Type of funding sources: None.
Dyspnea and perfusion defects (V/Q scan) are common after an acute pulmonary embolus whereas chronic thromboembolic pulmonary hypertension (CTEPH) is a rare sequele
Authors: Andersson T , Nilsson L , Larsen F , Carlberg B , Soderberg S 
Published: October 2022

Abstract

Introduction
Acute pulmonary embolism (PE), a common disease with substantial morbidity and mortality. Many previous studies have focused on the short-term consequences after an acute PE, but less attention has been directed to the long-term effects of a PE. The most feared complication is chronic thromboembolic pulmonary hypertension (CTEPH), with reported incidence rates ranging from 0.6 to 4.8%. Even though CTEPH is a rare complication after PE, dyspnea and functional impairment are common complaints, and a syndrome named PPES (post-PE-syndrome) has been suggested. The aim of this study was to follow a large national-wide cohort of post-PE-patients and to gather information regarding long-term symptoms and to determine the prevalence of CTEPH.

Patients and methods
We identified all patients diagnosed with acute PE in 2005 (ICD-10-SE: I26.0 and I26.9) that were registered in the Swedish National Patient Register (NPR), which has nearly 100% coverage.
In 2007, all surviving individuals were invited to participate by letter. They were asked to complete a questionnaire regarding symptoms and risk factors for long-term sequele including CTEPH.
Individuals who reported dyspnea or had known risk factors for CTEPH were invited to a follow-up with blood tests (NT-pro-BNP) and pulmonary scintigraphy. If any perfusion defects were detected, an echocardiography was performed. If signs of pulmonary hypertension, a referral was done to the regional PAH-center.

Results
In 2005, 5793 unique patients were admitted with a PE diagnosis in Sweden. Of those, 3510 were still alive in 2007, and 3226 individuals were contacted and asked to complete the questionnaire. Altogether 2105 (65%) individuals returned the questionnaire, and 53% reported dyspnea and 15% were asymptomatic but had risk factors for CTEPH. Altogether 944 individuals were referred for further follow-up including a recommendation to perform a V/Q-scan. Altogether 530 V/Q-scans were performed and 44% showed perfusion defects, whereof 85% had an echocardiography. Twenty-seven % of them registered a systolic right ventricular pressure of at least 30 mmHg. Referrals resulted in 6 cases with proved CTEPH. In parallel, 10 patients with CTEPH diagnosed before 2005 were detected, and 8 patients were diagnosed after 2005 and outside the study.Altogether 24 patients with CTEPH were thus identified and the cumulative incidence of CTEPH ranged between 0.7 and 1.2%.

Conclusion
Dyspnea is common after an acute PE and over 40% of them have perfusion defects on a V/Q scan, suggesting the presence of a post PE-syndrome (PPES). The incidence of CTEPH is however low. More research is needed to better define PPES.

Funding Acknowledgement
Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Actelion PharamceuticalsSwedish Heart and Lung Foundation