bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical significance of controlling nutritional status score for predicting short-term clinical events in takotsubo syndrome: a multicenter study
Authors: Takahashi K , Sudo M , Ogaku A , Saito Y , Atsumi W , Sonoda K , Nomoto K , Tachibana E , Okumura Y 
Published: November 2020

Abstract

Background
The Controlling Nutritional Status (CONUT) score is well known as a marker of nutritional status. Previous studies have reported that CONUT score could predict a prognosis of acute or chronic heart failure, and infective endocarditis. Takotsubo syndrome (TTS) is said to have a relatively good prognosis, but some patients have a bad turning point in hospital stay. Lower systolic blood pressure on admission, history of diabetes mellitus, and β-blocker use before admission have been reported as predictors of in-hospital cardiac complications. However, the prognostic utility of CONUT score in TTS is unclear. The aim of study was to evaluate duration of hospital stay and short-term clinical events with CONUT score in TTS.

Methods
Seventy-nine TTS patients who were admitted to 3 medical centers in Japan between January 2011 and October 2019 were enrolled. The average age was 71.8±11.5 years old, and the prevalence of female sex was 81%. The CONUT score was calculated based on the serum albumin, total lymphocyte and total cholesterol on admission. We retrospectively investigated the association between the short-term clinical events and CONUT score. The duration of hospital stay was defined as the primely outcome, and all cause death and congestive heart failure in hospital stay as the secondary outcome.

Results
The average CONUT score was 3.7±3.0. A positive correlation was found between the CONUT score and the duration of hospital stay (r=0.56, p&lt;0.01). Twenty (25.3%) patients suffered from clinical events (all cause death and congestive heart failure in hospital). Those patients with clinical events had significantly higher the CONUT score than those without (all cause death, 7.2±2.6 vs. 3.5±2.9, p&lt;0.01, congestive heart failure, 5.3±3.4 vs. 3.3±2.8, p=0.02, composite clinical events, 5.8±3.2 vs. 3.0±2.6, p&lt;0.01). ROC curve analysis revealed that the optimal cut-off value of the CONUT score for the prediction of composite clinical events was 4.0 (AUC: 0.75, sensitivity: 80%, Specificity: 64%). The patients with CONUT score of 4 or more (high COUNT score) were more prevalent in patients who experienced composite clinical events than in those who didn't (80% vs. 35.6%, p&lt;0.01). The patients with a high CONUT score had a longer hospital stay and higher occurrence of composite clinical events than those with CONUT score less than 4 (respectively, 27.2±19.1 days vs. 13.8±8.3 days, p&lt;0.01, 25.3% vs. 9.5%, p&lt;0.01).

Conclusions
The CONUT score in TTS patients was strongly associated with the duration of hospital stay and clinical events in hospital. The CONUT score is a simple indicator that can be calculated with only three factors. Therefore, the CONUT score on admission may be useful for a predictor of short-term clinical events in TTS patients.

Funding Acknowledgement
Type of funding source: None
The contribution of X-chromosome genomic imprinting to the bicuspid aortic valve and aortic coarctation prevalence in women with Turner syndrome
Authors: Klaskova E , Vrtel P , Vrtel R , Adamova K , Vrbicka D.I.T.A , Zapletalova J , Prochazka M , Pavlicek J , Lebl J , Stara V , Soucek O , Snajderova M , Hana V 
Published: November 2020

Abstract

Introduction
Turner syndrome (TS) is caused by the absence or structural abnormality of X chromosome. Compared with the general population, the prevalence of congenital heart defects is significantly higher in women with TS, especially with 45,X karyotype. Moreover, congenital heart defects represent the major risk for aortic dissection in TS individuals.

Purpose
There is a lack of reliable evidence whether the extremely variable cardiovascular phenotype including presence of aortic coarctation (CoA) and bicuspid aortic valve (BAV) in TS women may be influenced by the parental origin of the retained X chromosome.

Methods
DNA samples were collected from peripheral lymphocytes of 48 women with non-mosaic 45,X karyotype and from buccal swab of their biological parents' cheek. Subsequently, the single normal X-chromosome origin was identified. Based on genetic evaluation, patients were divided into two subgroups according to the parenteral original of X chromosome - maternal (XM), and paternal (XP). Complete cardiovascular examination (echocardiography, MRI of the heart and great vessels) was performed in each of our study patient. Differences between the prevalence of BAV and CoA in two above mentioned subgroups were tested by Student's t-test using R Statistical Software version 2.15.3.

Results
The origin of the single X chromosome was as follows: in 14 (29.2%) individuals was proved paternal and in 34 (70.8%) maternal origin of X chromosome. The prevalence of BAV in the whole group was 47.9%, in XP 58%, in XM 44.1%; the prevalence of CoA in the whole group was 8.3%, in XP 7.1%, in XM 8.8%. There was no statistically significant difference identified between the prevalence of BAV and CoA in XP and XM subgroups.

Conclusions
Our study confirmed an extremely high prevalence of BAV and CoA in non-mosaic women with X chromosome monosomy but no clear evidence for X-linked genomic imprinting effect on the CoA and BAV development in TS individuals was found. However, further studies of larger numbers of TS patients are crucial to finally clarify the real relationship of genomic imprinting and cardiovascular disease in TS.

Funding Acknowledgement
Type of funding source: Public grant(s) – National budget only. Main funding source(s): Supported by the grant from Ministry of Health of the Czech Republic VES 2017
Risk of myocardial infarction in middle aged and older patients with coarctation of the aorta
Authors: Fedchenko M , Mandalenakis Z , Giang K.W , Eriksson P , Rosengren A , Dellborg M 
Published: November 2020

Abstract

Introduction
Previous case-control studies have compared the risk of coronary artery disease/myocardial infarction (MI) in patients with coarctation of the aorta (CoA) with other congenital heart disease diagnoses, however, these studies have only included younger patients in their 20s and 30s, not older patients. As the atherosclerotic burden is increasing with increasing age, it is important to study the risk of MI in older patients (from early middle age and older) with CoA.

Purpose
The aim of our study was to investigate the risk of MI in older patients (≥40 years) with CoA, and to compare this risk with the risk in patients of the same age with ventricular septal defects (VSD), the most common congenital heart condition.

Methods
We used data from the Swedish National Patient Registry (NPR) to identify all patients alive at 40 years of age with a diagnosis of CoA or VSD, born during the period 1930–1970. The follow-up through the NPR and the Cause-specific Death Registry started in January 1970 and went on until December 2017.

Results
Altogether 1204 patients with CoA and 2079 patients with VSD were identified in the registers, and in total, 97 (8.1%) patients with CoA and 162 (7.8%) patients with VSD developed an MI during follow up. Mean follow up time was 20.6±10.3 years in CoA patients and 21.3±10.9 in the VSD group. The risk of MI was similar in CoA compared with VSD patients (HR 1.1, 95% confidence interval 0.9–1.5, p=0.3) Median age at MI was similar in CoA patients and in VSD patients; in CoA 59.8 years (range 40.1–87.5), in VSD 61.2 (range 40.2–87.5), p=0.3. Hypertension (diagnosed before MI or within a year after MI) was more common in CoA patients with an MI (58.8%) compared with VSD patients (37.7%), however, prevalence of diabetes mellitus and hyperlipidemia was similar in both patient groups (18.6% and 21.6% in CoA respectively, compared to 17.9% and 22.8% in VSD group). After adjustment for hypertension, diabetes mellitus and hyperlipidemia, the risk of MI remained similar in patients with CoA and VSD (hazard ratio 1.1, 95% confidence interval 0.8–1.4, p=0.5).

Conclusion
In this large nationwide study, we found that in older patients with CoA (median age at MI 60 years, range 40–88 years) the risk of MI was not increased and similar to that of patients with VSD with similar age at MI. Adjustment for hypertension, diabetes mellitus and hyperlipidemia did not modify this finding which suggests that patients with CoA do not have an increased risk of MI, compared to patients with VSD.

Funding Acknowledgement
Type of funding source: Public grant(s) – National budget only. Main funding source(s): This work was funded by the Swedish state under an agreement between the Swedish government and county councils, the ALF agreement (Grant number: 236611) and the Swedish Heart-Lung Foundation (Grant Number: 20090724).
Early experience with selexipag for the treatment of adults with pulmonary arterial hypertension associated with congenital heart disease
Authors: Van Dissel A , Post M , Sieswerda G.T , Vliegen H.W , Van Dijk A.P.J , Duijnhouwer A.L , Mulder B.J.M , Bouma B.J 
Published: November 2020

Abstract

Background
Recently, selexipag, a new orally available and selective prostacyclin receptor agonist, has become available for treatment of pulmonary arterial hypertension (PAH), but experience in patients with PAH associated with congenital heart disease (CHD) is limited to patients with closed defects.

Purpose
We present our early multi-centre experience using selexipag in the heterogeneous PAH-CHD population.

Methods
We prospectively evaluated adults with PAH-CHD from five PAH-CHD expert centres who were treated with selexipag. Patients were titrated to highest tolerable individualized dose (200 to 1,600 μg twice daily), after which patients entered the maintenance phase. Data on functional class (FC), 6-minute walk distance (6MWD), imaging and biochemical (N terminal pro-brain natriuretic peptide [NT-proBNP]) parameters were collected.

Results
Thirty-four patients (age 43±14 years, 56% female, 60% Eisenmenger syndrome, 22% Down syndrome, 60% dual PAH therapy) were started on selexipag. All patients experienced at least 2 side effects during the initial uptitration phase. Most side effects were manageable and diminished after reaching the maintenance dose, but eight patients discontinued treatment due to side effects during the titration phase. The most frequent side effects were consistent with the known side effects of prostacyclins, including headache, nausea, diarrhoea and jaw pain. Majority (68%) of patients reached lower maintenance doses of 200–600 μg. At 12 months, FC improved in three patients and remained unchanged in the others. 6-minute walk distance remained stable throughout follow-up (475 to 470 m; p=n.s.) in patients who remained on-treatment compared to patients who stopped selexipag (485 to 370 m). NT-proBNP levels remained stable in patients on-treatment (520 to 600 ng/L) but worsened in patients who stopped (700 to 1000 ng/L). One patient died during follow-up from end-stage heart failure.

Conclusion
There is a promising role for selexipag in the treatment of adults with PAH-CHD. However, based on our experience, the use is challenging due to complexity in dosing and side effect profiles, which limit patients' tolerability and acceptance during the titration phase.

Funding Acknowledgement
Type of funding source: Other. Main funding source(s): Actelion Pharmaceuticals
Comparison between cheatham-platinum stent and self-expandable uncovered nitinol stent in patients with coarctation of aorta – a randomized controlled trial
Authors: Sadeghipour P , Shafe O , Moosavi J , Basiri H.A , Abdi S , Firouzi A , Khajali Z , Saedi S , Mohebbi B , Maleki M , Pouraliakbar H.R , Samiei N , Sadeghpour A , Alemzadeh M.J , Shahdi S 
Published: November 2020

Abstract

Background
Endovascular treatment is considered the main therapeutic approach for native coarctation of aorta (CoA) in adult patients. Balloon-expandable stents (BE) have been introduced as the primary choice for coarctoplasty. However during the recent years, self-expandable (SE) stents with acceptable radial force have been suggested as an alternative. Still, no randomized controlled (RCT) trials have compared the efficacy and safety of both strategies.

Methods
The present study is a prospective, single center, parallel-group, open-label RCT on patients with native CoA (Figure). Patients were randomized into Bare Cheatham-Platinum Stents and Uncovered Nitinol Stent (SE). All patients were scheduled to be followed in 1-, 6-, 12-month and 36-month intervals. The present report, focuses on the one-year outcome of the study.

Results
Of 101 patients with native CoA referred to our center, 93 patients have been randomized into BE- (47 patients) and SE-arm (46 patients). One year follow up is scheduled to be completed on March 2020. In the preliminary analysis of 62 patients, 1 intraprocedural stent migration has occurred in each group. No aortic perforation was detected in the study. Vascular access site complications were similar between the two groups (3 (6.3%) in BE versus 1 (2.1%) in SE, p value= 0.432). One nonfatal stroke occurred in BE group. No mortality or bailout surgical repair happened in one year study in the two groups. One patient with re-coarctations were detected during one year follow up in each of the groups (p value=0.210). No aortic pseudoaneurysm or aneurysmal formation were observed in 12-month follow up aortic CT angiography.

Conclusion
The preliminary results showed no significant difference in efficacy and safety of both treatment modalities. The final result is pending.
Study Flow Chart

Funding Acknowledgement
Type of funding source: None
Anatomical predictors of residual left-to-right shunt after percutaneous suture-mediated patent fossa ovale closure
Authors: Gaspardone A , De Santis A , Iamele M , D'Ascoli E , Piccioni F , D'Errico F , Giannico M.B , Summaria F , Gioffre' G , Sgueglia G 
Published: November 2020

Abstract

Background
Percutaneous suture-mediated patent fossa ovale (PFO) closure has recently been introduced in clinical practice to overcome most of the limitations of metallic PFO occluders with early results showing a favourable efficacy and safety profile in most PFO cases.

Purpose
To assess PFO anatomy in a large series of patients undergoing percutaneous suture-mediated PFO closure in order to identify anatomical predictors of significant residual right-to-left shunt (RLS) and thus appropriately select patients to be submitted to this technique.

Methods
Pre-procedural transesophageal echocardiogram of 230 patients undergoing suture-mediated PFO closure at our Institution were carefully reviewed both qualitatively and quantitatively. The following parameters were systematically assessed in all reviewed cases: presence and grade of baseline atrial RLS, presence of bidirectional shunt, PFO length and width, presence of atrial septal aneurysm and its maximal bulge, presence and size of an embryonic or foetal remnant.

Results
In 37 patients a residual atrial RLS ≥2 grade was found at a mean follow-up of 248±147 days. The following variable were found to be significantly associated with significant residual atrial RLS at follow-up: grade of baseline spontaneous and Valsalva manoeuvre RLS shunt (odds ratio 2.39, 95% confidence interval 1.48–3.89, p=0.001 and odds ratio 3.07, 95% confidence interval 1.22–7.23, p=0.017), baseline bidirectional shunt across the PFO (odds ratio 3.59, 95% confidence interval 1.47–8.77, p=0.005) and PFO width (odds ratio 2.61, 95% confidence interval 1.92–3.55, p=0.001).
At multivariable analysis, only PFO width ≥5 mm (odds ratio 10.97, 95% confidence interval 4.22–28.56, p=0.001) and grade of baseline RLS shunt (odds ratio 1.84, 95% confidence interval 1.05–3.21, p=0.032) were independent predictors of a significant atrial RLS at follow-up.

Conclusions
Suture-mediated PFO closure represents a valid alternative to traditional devices with an excellent safety and efficacy profile at follow-up. As for any new technique, it is extremely important to select the right anatomical and functional features predictive of a successful closure. The results of this study indicate that the suture-mediated closure of PFO is feasible in the majority of septal anatomies however wide PFO ≥5 mm are less likely to be closed with only one stitch.

Funding Acknowledgement
Type of funding source: None
Pulmonary vascular resistance and pulmonary artery compliance in a large cohort of patients with sinus venosus septal defect
Authors: Van De Bruaene A , Van Dessel L , Troost E , De Meester P , Budts W 
Published: November 2020

Abstract

Background
Left-to-right shunt in patients with sinus venosus septal defect (SVSD) may affect resistive (pulmonary vascular resistance – PVR) and elastic (pulmonary artery compliance-PAC) properties of the pulmonary arteries. This study aimed at evaluating (1) changes in hemodynamics over time and (2) the relationship between PVR and PAC in a large cohort of patients with SVSD.

Methods
Data of 136 patients with SVSD (median age at diagnosis 14 (IQR 5–48) years, 47% male) were included. Catheterization data was available in 87 patients. Pressures were measured and cardiac output evaluated using the Fick principle. PVR (mean pulmonary artery pressure (mPAP)-wedge pressure/cardiac output) and PAC (stroke volume/pulse pressure) were calculated and indexed for body surface area.

Results
Ninety percent had abnormal pulmonary venous connection. Median shunt ratio was 2.5 (IQR 2.0–2.9). Repair was performed in 128 (94%) at a median age of 13 (IQR 5–43) years. During a median follow-up time of 31 (IQR 17–55) years, 12 (9%) patients died, 13 (10%) developed heart failure, 19 (14%) atrial arrhythmia, 6 (4%) sick sinus syndrome and 7 (5%) required pacemaker implantation. PVR indexed and PAC indexed was 3.5 (IQR 2.4–7.5) WU·m2 and 1.8 (IQR 1.3–2.5) mL/mmHg·m2. There was an inverse, hyperbolic relationship between PVR and PAC with an RC time of 6.6 (IQR 4.4–8.9) sec. When comparing patients with age at catheterization &lt;10 years, 10–50 years and &gt;50 years, shunt ratio (2.5±0.6 vs 2.5±0.8 vs 2.5±0.6; p=0.836) and PVR indexed (2.9 (2.3–3.4) vs 3.1 (2.0–9.0) vs 6.6 (3.6–8.8) WU·m2; p=0.132) were not statistically different, whereas mPAP (19 (16–22) vs 21 (16–24) vs 28 (22–34) mmHg; p=0.002); wedge (6 (4–8) vs 13 (8–16)vs 14 (10–19) mmHg; p&lt;0.0001) and PAC indexed (2.2 (1.5–2.9) vs 2.0 (1.4–2.8) vs 1.3 (0.9–1.7) mL/mmHg·m2; p=0.002) differed significantly.

Conclusion
SVSD is associated with late morbidity and mortality. In SVSD, PVR and PAC are inversely related. Over time, the changes in elastic resistance (PAC or pulsatile load) are more profound than changes in resistive (PVR – steady load).
Figure 1

Funding Acknowledgement
Type of funding source: None
Outcome of patients with tetralogy of Fallot repair over 50 years of age
Authors: Antal A , Szegedi M , Takacs E , Szabo B , Solymosi B , Vincze V , Ablonczy L , Kornyei L , Temesvari A , Andreka P , Balint H 
Published: November 2020

Abstract

Purpose
To report the outcomes of patients with repaired tetralogy of Fallot (TOF) above 50 years of age.

Methods
We reviewed records of TOF patients who were followed in our institution since 2000. Demographic data, surgical reports and long-term outcomes were analyzed retrospectively.

Results
Thirty-five of 297 (12%) patients who underwent TOF repair were above 50 years of age (25 men [60%]) at the last follow-up. Eighteen patients (48%) had prior palliative surgery. The mean age at the first repair was 18±13 years (5 patients after 40 years of age). Reoperation with pulmonary valve replacement was performed in 10 (28%) patients, and a second redo surgery in 6 (17%) patients. During a mean 17± 2 years follow-up 7 (8.5%) patients died (n=6 end-stage heart failure, n=1 infective endocarditis). At the last follow-up 21/35 patients (60%) were treated with tachyarrhythmia, 19/35 (54%) with chronic heart failure and 17/35 (48%) with comorbidities (hypertension, coronary artery disease/peripherial artery disease, diabetes mellitus, renal failure). Using multivariate analysis the only predictor of death was heart failure (OR: 6.8). Furthermore atrial tachycardia (OR: 8.8) and at least grade II tricuspid regurgitation (OR: 5.0) were identified as predictors of heart failure.

Conclusion
This historical cohort of TOF patients with late repair has a high morbidity rate later in life. Approximately half of the patients require treatment for chronic heart failure, arrhythmias and cardiovascular related comorbidities. Atrial tachycardia and tricuspid regurgitation are strong predictors for heart failure. In this population the strongest predictor for death is heart failure.

Funding Acknowledgement
Type of funding source: None
Right atrial mechanics by strain echocardiography in chronic thromboembolic pulmonary hypertension
Authors: Ferreira Fonseca M , Baptista R , Samouco G , Soeiro P , Martins R , Castro G , Goncalves L 
Published: November 2020

Abstract

Introduction
Chronic thromboembolic pulmonary hypertension (CPTEH) is the result of thromboemboli leading not only to mechanical obstruction but also pulmonary vascular remodelling and progressive increase in right heart afterload. Prognostic assessment in CPTEH is complex and multifactorial.

Purpose
We used two-dimensional strain echocardiography (2D-STE) to quantify right atrial (RA) mechanics and its correlation with invasive hemodynamics, load-dependent biomarkers and well-known prognostic markers in patients with CTEPH.

Methods
A total of 44 patients (24 females, mean age 61±15.6 years) with CTEPH were recruited. 2D-STE was used to measure right atrial reservoir strain (RASr) (Figure 1) which was then compared to conventional ultrasound measurements, right heart catheterisation (RHC) measurements, the percentage of obstruction in ventilation-perfusion pulmonary single-photon emission computed tomography (V/Q SPECT), B-type natriuretic peptide (BNP) values and 6-minute walk test (6MWT) performance. All patients underwent transthoracic echocardiographic evaluation with a maximal time distance of three months to RHC.

Results
There was a significant positive correlation of RASr with RV longitudinal function determined by tricuspid annular plane systolic excursion (TAPSE) (r=0.488, p=0.003), tricuspid annular peak systolic velocity (S') (r=0.490, p=0.002), right ventricular outflow tract velocity time integral (RVOT VTI) (r=0.457, p=0.005), as well as with 6MWT (r=0.491, p=0.004). There was a significant negative correlation of RASr with mean pulmonary artery pressure (r=−0.513, p=0.002), pulmonary vascular resistance (r=−0.439, p=0.011) and right atrial pressure (RAP) (r=−0.513, p=0.002). RASr was also correlated with Log-transformed BNP values (r=−0.552, p&lt;0,001), and in a multivariate linear regression model, RASr was an independent predictor of Log-transformed BNP values (β=−0.448, 95% CI: −0.046 to −0.009; p=0.005). There was no correlation between RASr and the percentage of obstruction in V/Q SPECT (r=0.164, p=0.388).

Conclusion
2D-STE-derived RA mechanics demonstrated to be a useful, non-invasive, surrogate measurement of RHC parameters. It also predicted important clinical/laboratory prognostic measurements, such as BNP and 6MWT performance. The absence of correlation between 2D-STE values and the percentage of vessel obstruction determined by SPECT may suggest that RV maladaptive response to the obstruction, rather than the degree of obstruction, dictates right heart failure in CTEPH.
Right atrial strain components

Funding Acknowledgement
Type of funding source: None