bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical course long after atrial switch: a novel risk score for serious clinical events
Authors: Woudstra O , Zandstra T.E , Vogel R.F , Van Dijk A.P.J , Vliegen H.W , Kies P , Jongbloed M.R.M , Egorova A.D , Doevendans P.A , Konings T.C , Mulder B.J.M , Tanck M.W.T , Meijboom F.J , Bouma B.J 
Published: November 2020

Abstract

Background
Patients after atrial switch surgery for transposition of the great arteries (TGA-AtrSO) experience serious clinical events during adulthood, mainly heart failure and arrhythmias, but data on the emerging risks remain scarce.

Purpose
To assess the risk for events during the clinical course in adulthood of TGA-AtrSO patients and provide a novel risk score for event-free survival.

Methods
We reviewed medical records of TGA-AtrSO patients from five hospitals. Endpoints were all-cause mortality, heart failure (HF), defined as HF hospitalizations, heart transplantation, ventricular assist device implantation, or HF-related death, and symptomatic ventricular arrhythmias (VA). Predictors for event-free survival were examined to construct a prediction model using bootstrapping techniques.

Results
We followed 169 TGA-AtrSO patients (60% Mustard, age 28 [IQR 24–36] years) for 13 [IQR 9–16] years, during which 17 (10%) died, 34 (20%) had HF events, and 15 (9%) had VA events. Five-year risk of mortality, first HF event, and first VA increased from 1% each at age 25, to 7% (95% CI 4–10%), 17% (95% CI 10–25%), and 4% (95% CI 2–8%), respectively, at age 50. A prediction model combining age &gt;30, prior VA, age &gt;1 year at repair surgery, QRS duration &gt;120ms, ≥mild LV dysfunction, and severe tricuspid regurgitation discriminated well between patients at low (&lt;5%), medium (5–20%) and high (&gt;20%) 5-year risk (optimism corrected C-statistic=0.84). Observed 5- and 10-year survival in low-risk patients were 100% and 99%, compared to only 45% and 19% in high-risk patients.

Conclusion
The clinical course of atrial switch patients increasingly consists of serious clinical events, especially heart failure. A novel risk score stratifying patients as low, medium, and high risk for event-free survival is presented, providing information on absolute individual risks which may support decisions for pharmacological and interventional management.
Figure 1. Observed event-free survival of patients with predicted low risk (&lt;5% in 5 years), medium risk (5–20% in 5 years) and high risk (&gt;20% in 5 years).

Funding Acknowledgement
Type of funding source: Foundation. Main funding source(s): Dutch Heart Foundation; Amsterdam University Fund
Decreasing mortality in patients with simple congenital heart disease: a Danish nationwide study
Authors: El-Chouli M , Malmborg M , Bang C.N.F , Gislason G.H 
Published: November 2020

Abstract

Background
The long-term mortality in patients with simple congenital heart disease (SCHD) compared with the general population is not well-described.

Purpose
To investigate the 10-year mortality in individuals with and without SCHD and whether it has changed since 1977 using contemporary data.

Method
By linking Danish nationwide registries, we identified all individuals with and without a SCHD diagnosis who were alive at age 40 between 1977–2006. Excluded were individuals with moderate or severe congenital heart disease. SCHD was defined as isolated ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA) or pulmonary stenosis (PS). The population was followed from age 40 until death or emigration, whichever came first. We predicted 10-year all-cause mortality according to each year of inclusion. Reported was 10-year all-cause mortality and mortality ratios (SCHD vs non-SCHD) with 95% confidence intervals (CI) by calendar year groups (1977–1986, 1987–1996, 1997–2006).

Results
We identified 2,040 individuals with SCHD (VSD: 27.5%, ASD: 62.2%, PDA 6.8%, PS: 3.5%), of which 1,121 (55.0%) were female, and 2,083,277 individuals without SCHD, of which 1,028,769 (49.4%) were female. In individuals with SCHD the 10-year all-cause mortality decreased over time in both men (1977–1986: 12.3% [11.8–12.9%], 1987–1996: 9.0% [7.4–10.5%], 1997–2006: 5.0% [4.3–5.7%]) and women (1977–1986: 7.7% [7.5–7.9%], 1987–1996: 4.9% [3.9–6.0%], 1997–2006: 1.2% [0.7–1.7%]), whereas the 10-year risks were somewhat stable in individuals without SCHD for both men (1977–1986: 3.2% [3.2–3.2%], 1987–1996: 3.3% [3.2–3.3%], 1997–2006: 2.9% [2.7–3.0%]) and women (1977–1986: 2.4% [2.3–2.4%], 1987–1996: 2.1% [2.1– 2.2%], 1997–2006: 1.7% [1.6–1.8%]) (Figure 1, panel A). The mortality ratio decreased over time in both men (1977–1986: 3.9 [3.7–4.1], 1987–1996: 2.7 [2.3–3.2], 1997–2006: 1.7 [1.5–1.9]) and women (1977–1986: 3.3 [3.2–3.3], 1987–1996: 2.3 [1.8– 2.7], 1997–2006: 0.7 [0.4–1.0]) (Figure 1, panel B) remaining significantly higher for men, but not women, in 1997–2006.

Conclusion
In individuals with simple congenital heart disease aged 40 years, the 10-year mortality decreased dramatically over time for both men and women. Despite decreasing mortality, men with SCHD, but not women, remained at a higher 10-year mortality compared to individuals without SCHD.
Figure 1. Temporal trends in 10-year mortality

Funding Acknowledgement
Type of funding source: Foundation. Main funding source(s): Danish Heart Foundation
Echocardiography and cardiac magnetic resonance in children with repaired tetralogy of fallot and their correlations with exercise capacity
Authors: Avesani M , Borrelli N , Krupickova S , Sabatino J , Piccinelli E , Josen M , Michielon G , Fraisse A , Di Salvo G 
Published: November 2020

Abstract

Background
Severe pulmonary regurgitation (PR) and progressive right ventricular (RV) dilation and disfunction are common in patients with repaired Tetralogy of Fallot (r-TOF) and should be carefully monitored during the follow up of these patients. In this contest, Echocardiography and Cardiac Magnetic Resonance (CMR) have a complementary diagnostic role.

Purpose
To correlate Echo and CMR parameters in children (&lt;18 years) with r-TOF with at least moderate PR assessed by Echocardiography and to analyse which parameter was associated with peak oxygen consumption (Vo2).

Methods
Paediatric patients with r- TOF with at least moderate PR at the echo evaluation who underwent a CMR study within six months were included by using hospital databases. All patients underwent standard echo-Doppler study including RV end-diastolic area (RVEDA), end-systolic area (RVESA), fractional area change (FAC) and TAPSE; PR was assessed by Color Doppler, continuous-wave (CW) Doppler and derived parameters such as pressure half time (PHT), PR index, ratio of diastolic and systolic time-velocity integrals (DSTVI) of the main pulmonary artery. By speckle tracking we measured also RV global longitudinal strain (RVGLS) and right atrial strain (RAS). All the patients underwent CMR to assess PR and right ventricular volumes and ejection fraction (EF). Of these, 36 patients underwent cardiopulmonary exercise test (CPET).

Results
Fourty-six children (aged 13.7±3.0 years) were included. Echo derived RV areas correlated significantly with CMR RV volumes (r=0.72, p&lt;0.0001). RVEDA &gt;21.9 cm2/m2 had a good sensitivity (83.3%) and specificity (73.5%) to identify a RV end-diastolic volume (RVEDV) ≥150 ml/m2. No correlation was found among TAPSE, FAC, RVGLS and RVEF calculated by CMR nor between PHT, PR index and DSTVI and PR-RF. Only A' wave velocity showed a significant but modest correlation with CMR RF (r=0.57, p&lt;0.0001). Flow reversal in pulmonary branches showed a sensitivity of 95.8% and a specificity of 59.1% to identify PR RF ≥35%. RVEF by CMR was preserved in all patients, while TAPSE was reduced in 78.2% and RVGLS in 60.8%. None of the CMR parameters correlated with peak Vo2. At the multivariate analysis RAS was the best independent predictor of peak Vo2 (p&lt;0.0001).

Conclusion
In children, flow reversal in pulmonary branches identifies hemodynamically significant PR at CMR. RV area by echocardiogram is a valid first-line parameter to screen RV dilation. Our study suggests that, also for the RV, there is longitudinal systolic dysfunction in presence of preserved RV EF. RAS is the best predictor of peak Vo2 and should be added in the follow up of these patients.

Funding Acknowledgement
Type of funding source: None
Late gadolinium-enhanced cardiac magnetic resonance in repaired tetralogy of Fallot: characterization of the right ventricular arrhythmogenic substrate
Authors: Rodriguez Garcia J , Pijuan Domenech A , Pascual Gonzalez G , Dos Subira L , Perez Rodon J , Benito Villabriga B , Francisco Pascual J , Santos Ortega A , Subirana Domenech M , Miranda Barrio B , Gordon Ramirez B , Cuellar Calabria H , Valente F , Ferreira Gonzalez I , Rivas Gandara N 
Published: November 2020

Abstract

Introduction
Ventricular arrhythmias are one of the main causes of morbidity and mortality in patients with repaired tetralogy of fallot (rTF). These life-threatening arrhythmias are related to specific isthmuses of viable tissue between areas of scar and/or valve rings of the right ventricle (RV) that have been already described in the literature. Late gadolinium-enhanced cardiac magnetic resonance (LGE-CMR) has proven useful in characterizing the arrhythmogenic substrate in several heart diseases. Nevertheless, LGE-CMR evidence in patients with rTF is scarce.

Purpose
To compare the characterization of the ventricular arrhythmogenic substrate by means of LGE-CMR and electroanatomic mapping (EAM) of the right ventricle (RV) in patients with rTF.

Methods
Unicentric and observational study of consecutive patients with rTF who underwent LGE-CMR performed with 1.5T equipment and RV high-density voltage map, performed with multipolar catheter and completed with contact force-sensing catheter. The LGE-CMR segmentation was performed with dedicated software. The extent (area and percentage) and location of the dense scar (defined as &lt;0.5 mV in EAM and &lt;40% of the maximum intensity pixel in LGE-CMR) were compared, as well as the location of the isthmuses of viable tissue.

Results
Eight patients were included (45.7±10.4 years; 50% male). The extent of the scar was 20.7±13.6 cm2 (15.1±9.3%) by EAM and 21.7±8.8 cm2 (11.8±7.6%) by LGE-CMR. There was an absolute correlation regarding the location of the dense scar and the distribution of the isthmuses of viable tissue. The quantification of the dense scar evidenced a positive linear correlation between both techniques (area correlation: ρ = 0.71, p=0.047; percentage correlation: ρ = 0.88; p=0.004). The average time spent for the segmentation of the LGE-CMR with the dedicated software was 19.9±3.0 minutes.

Conclusions
Characterization of the RV arrhythmogenic substrate in patients with rTF with LGE-CMR is feasible. An absolute association regarding the location of the dense scar and the distribution of the isthmuses of viable tissue was observed when compared to the RV high-density EAM. In the same way, a statistically significant linear correlation in the quantification of the dense scar between both techniques was documented.
LGE-CMR and EAM of two patients with rTF

Funding Acknowledgement
Type of funding source: None
T1 mapping to assess hepatic and myocardial characteristics in children with single ventricle circulation
Authors: Beigh M , Pagano J.J , Noga M , Harake D , Olugbuyi O , Tham E 
Published: November 2020

Abstract

Background
Single ventricle (SV) palliation, culminating with the Fontan operation, results in passive systemic venous blood flow directly to the pulmonary circulation. Resulting inevitable hepatic venous congestion can lead to hepatic fibrosis. Previous studies suggest hepatic changes can occur prior to the Fontan completion. Besides fibrotic myocardial remodeling may lead to systolic and diastolic ventricular dysfunction, transmitting back pressure to the pulmonary system.

Purpose
To compare quantitative T1 cardiovascular magnetic resonance (CMR) imaging of the myocardium and liver between SV patients and controls, as a potential measure of myocardial and hepatic fibrosis.

Methods
Retrospective review of 16 SV patients with dominant single left ventricle (SLV, n=6) or single right ventricle (SRV, n=10), at various stages of palliation (pre-Glenn=6, post-Glenn=3, Fontan=7) underwent CMR with myocardial T1 mapping with the liver also in the plane of view. Biventricular patients found to have structurally normal hearts and normal cardiac function on CMR were used as controls (n=21). Native T1 times using a modified Look-Locker inversion recovery (MOLLI) approach in free-wall of the dominant ventricle at a mid-ventricular short axis in SV and the ventricular septum in controls and, a region of interest in the liver (avoiding any vessels) were measured in all patients. Median and inter-quartile ranges of continuous variables were compared between SV and controls using the Mann-Whitney U test.

Results
As compared to controls SV patients were (1) significantly younger, (2) had lower ejection fraction, (3) higher median myocardial T1, and (4) higher median liver T1. Also, there was no difference between SLV vs. SRV median myocardial T1 (1056 vs. 1065ms, p=0.43) or liver T1 (678 vs. 729ms, p=0.30)

Conclusion
Despite younger age, findings of increased myocardial T1 may suggest an element of myocardial fibrosis responsible for the ventricular dysfunction in this population, and that raised liver T1 may be an earlier marker of liver fibrosis, which warrants further study.

Funding Acknowledgement
Type of funding source: None
Prevalence of arrhythmias on the long term of adult patients with partial anomalous pulmonary venous connection treated surgically and conservatively: data from the SACHER registry and a French center
Authors: Touray M , Bouchardy J , Ladouceur M , Schwerzmann M , Greutmann M , Tobler D , Harald G , Engel R , Pruvot E , Blanche C , Sekarski N , Rutz T 
Published: November 2020

Abstract

Introduction
Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital heart disease, characterized by one or some but not all pulmonary veins anomalously connected to the right atrium or a systemic vein. PAPVC is either an isolated shunt lesion or associated with an atrial septal defect (ASD). This study compares the arrhythmic outcome of patients treated surgically with those clinically monitored.

Method
Clinical, surgical, imaging and invasive data of PAPVC patients were retrospectively reviewed from 7 centers from the Swiss Adult Congenital HEart disease Registry (SACHER) and one French center.

Results
A total of 168 patients with PAPVC were identified. Most (77%) patients underwent surgery, while the remaining (23%) ones were treated conservatively with clinical monitoring. The operated group (OG) had a significantly higher number of associated ASD (N=106, 82%) (p&lt;0.001) and a higher number of anomalous pulmonary veins leading to a mean Qp:Qs at 2.5±1.2 before surgery (table). Moreover, the majority of patients in the OG had cardiac symptoms (N=78, 60%) and were diagnosed at a significantly younger age (table). Mean age at operation was 27±20 years.
Age did not differ at latest follow-up (table). Holter recordings revealed a higher prevalence of arrhythmia in the OG (p=0.031), mainly of supraventricular tachyarrhythmias (table). The occurrence of ventricular non-sustained tachycardia and of bradyarrhythmia did not statistically differ between groups. Patients in the OG required more often medical treatment for arrhythmias: 12 (9%) needed electrophysiological study in the OG and none in the NOG (p=0.057). The amount of patients requiring a pacemaker implantation in the OG (11%) was significantly higher than that of NOG (0%) (p=0.039).

Conclusion
Patients after PAPVC repair present with a significant higher burden of arrhythmia than conservatively treated patients, either due to a larger shunt pre-operatively and/or as a late complication of the corrective surgery itself.

Funding Acknowledgement
Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Unrestricted grant from Actelion, Switzerland
Long term outcome of single ventricle physiology with pulmonary restriction not undergoing Fontan repair
Authors: Buendia F , Ramirez B , Gallego P , Oliver J.M , Montserrat S , Osa A , Miranda B , Rodriguez Puras M.J , Gonzalez A.E , Carbonell B , Meras P , Adsuar A , Ruiz Cantador J , Rueda Soriano J , Dos L 
Published: November 2020

Abstract

Background
Patients with univentricular physiology who do not complete the palliation to Fontan are a heterogeneous group with unknown long term outcome.

Aims
This study aimed at describing the clinical course and long-term survival of patients with SV physiology with restricted pulmonary flow that had not undergone a Fontan type of repair.

Methods
From the prospectively maintained databases of the adult congenital cardiac units of five tertiary referral centers, data from all SV physiology patients were obtained. Patients completing a Fontan type palliation or developing Eisenmenger physiology and segmental pulmonary hypertension were excluded. Baseline data were recorded on the first visit at adult congenital heart disease (ACHD) unit. The primary end point was death.

Results
101 patients (50.5% females) were identified. Mean age at end of follow up was 39.3±11.3 years. Of these, 45 (44.6%) were unoperated (group 1, restricted forward pulmonary flow with or without pulmonary banding), 38 (37.6%) had undergone a cavopulmonary shunt as a definitive palliation (group 2) and 18 (17.8%) had aortopulmonary shunts (group 3). The main diagnosis was double inlet left ventricle (DILV) (N: 52, 51.5%) and most of the ventricle was left (82.2%). The principal reason for not performing a Fontan repair was mean pulmonary artery pressure &gt;18 mmHg. At initial visit at the ACHD unit patients were 32.2±11.1 years of age. 35% of the patients were in NYHA class III-IV, with no differences between groups. However, patients in group 2 had worse oxygen saturation (p=002) and higher haemoglobin (p=0.037). After a mean follow-up of 7.3±4.1 years, mortality was 20.8% (21 patients), being sudden death (7p, 6.9%) the most frequent cause. Patients in group 3 showed worse ventricular function (p=0.0001) and a trend to higher mortality that did not reach statistical significance (HR 2.7, CI 95% 0.91–8.14, P=0.07).

Conclusions
Patients with single ventricle physiology not undergoing Fontan repair are a population of high risk, with sudden death as main driver of mortality. Patients palliated with aortopulmonary shunts are prone to worse ventricular function and a trend to higher mortality.

Funding Acknowledgement
Type of funding source: Public hospital(s)
Left ventricular remodeling after transcatheter versus surgical therapy in adults with coarctation of aorta
Authors: Egbe A , Vojjini R , Banala K 
Published: November 2020

Abstract

Background
Transcatheter stent therapy is as effective as surgery in producing acute hemodynamic improvement in patients with coarctation of aorta (COA). However, left ventricular (LV) remodeling after transcatheter COA intervention has not been systematically investigated. The purpose of this study was to compare remodeling of LV structure and function after transcatheter stent therapy vs surgical therapy for COA.

Methods
LV remodeling was assessed at 1, 3 and 5-years post-intervention using: LV mass index (LVMI), LV end-diastolic dimension, LV ejection fraction, LV global longitudinal strain (LVGLS), LV e' and E/e'.

Results
There were 44 and 128 patients in the transcatheter and surgical groups respectively. Compared to the surgical group, the transcatheter group had less regression of LVMI (−4.6 [95% CI: −5.5 to −3.7] vs −7.3 [95% CI: −8.4 to −6.6] g/m2, p&lt;0.001), less improvement in LVGLS (2.1 [95% CI 1.8–2.4] vs 2.9 [95% CI: 2.6–3.2]%, p=0.024) and e' (1.0 [95% CI: 0.7–1.2] vs 1.5 [95% CI: 1.3–1.7] cm/s, p=0.009) at 5 years post-intervention. Exploratory analysis showed a correlation between change in LVMI and LVGLS, and between change in LVMI and e', and this correlations were independent of the type of intervention received.

Conclusions
Transcatheter stent therapy was associated with less remodeling of LV structure and function during mid-term follow-up. As transcatheter stent therapy become more widely used in the adult COA population, there is a need for ongoing clinical monitoring to determine if these observed differences in LV remodeling translates to differences in clinical outcomes.

Funding Acknowledgement
Type of funding source: None
Increased prevalence of left ventricular diastolic dysfunction in adults with coarctation of aorta
Authors: Egbe A , Vojjini R , Banala K 
Published: November 2020

Abstract

Background
Left ventricular (LV) pressure overload and coronary artery disease, both of which are common in patients with coarctation of aorta (COA), are risk factors for LV diastolic dysfunction. Patients with COA may have aortic vasculopathy that can result in LV pressure overload even in the absence of hemodynamically significant COA. We therefore hypothesized that patients with COA (without hemodynamically significant COA) will have more LV diastolic dysfunction compared to controls.

Methods
Adult patients with COA (OA peak velocity &lt;2 m/s) were matched 1:1 to patients without structural heart disease using propensity score method based on age, sex, body mass index, hypertension and blood pressure. The objective was to compare LV diastolic dysfunction (defined as E/e&gt;2 standard deviations above age-specific normative values) between COA patients and controls.

Results
Of 204 COA and 204 control patients (age 35±12 years), COA patients had higher septal and lateral E/e' ratio (12±4 vs 9±4, p=0.009) and (10±3 vs 7±3, p&lt;0.001), respectively. Compared to controls, the prevalence of LV diastolic dysfunction was higher in COA patients for every age group: &lt;40 years (63% vs 13%, p&lt;0.001); 41–60 years (87% vs 33%, p&lt;0.001); age &gt;60 years (82% vs 56%, p=0.076). Left ventricular mass index (LVMI) was the strongest determinant of E/e' (β=2.71 per 10 g/m2, standard error = 1.25, p&lt;0.001).

Conclusion
LV diastolic dysfunction was common in COA patients, and the association with LVMI suggests that COA patients may have ongoing LV pressure overload in the absence of hemodynamically significant re-coarctation.

Funding Acknowledgement
Type of funding source: None