bluuebunny/crossref_metadata_2025 huggingface dataset:
Long-term survival in patients with univentricular heart: a nationwide, register-based cohort study
Authors: Ozturk A G , Dellborg M , Giang K W , Dalen M , Sorensson P , Mandalenakis Z
Published: October 2022

Background

Univentricular heart (UVH) is a broad term that covers various cardiac structural abnormalities in which one ventricle is severely underdeveloped, or a ventricular septal wall did not form, leading to a functional single-ventricle physiology. Patients with UVH have a very limited life expectancy if not surgically treated. While the outcome in children with UVH have been extensively studied, data regarding the long-term outcomes in an unselected, nationwide cohort is still lacking.

Purpose

The aim of the present study was to determine long-term survival in patients with UVH compared to an age- and sex-matched control population without congenital heart disease in Sweden.

Methods

We linked data from the Swedish Patient and Cause of Death Register to identify patients with UVH that were born between 1970 and 2017. Each patient with UVH was matched with ten individuals without congenital heart disease for year of birth and sex. The mortality risk in patients with UVH compared with matched controls was analyzed using Cox proportional regression models and Kaplan-Meier survival analysis.

Results

Of a total of 5,075 patients with UVH and 50,620 matched controls, 1,992 (37.9%) and 485 (1.0%) died, respectively. The mean follow-up (standard deviation) was 15.7 (± 14.2) years. A total of 758 patients (14.9%) of the UVH population were diagnosed with hypoplastic left heart syndrome (HLHS). The mortality risk was 53.01 (95% CI, 48.0–58.6) times higher in UVH and 163.5 (95% CI, 124.3–215.2) times higher in HLHS compared to controls. According to birth period, highest mortality was found at the earliest UVH cohort (1970–1981) 59.8%, and decreased by later birth periods. The lowest mortality was in the latest birth period, 2006–2017 (16.3%).

Conclusions

In this nationwide, register-based cohort study, the overall risk of mortality was more than 50 times higher in patients with UVH compared to matched controls. However, an increased survival was observed over time and by later birth periods. Quadragenarians with UVH is still a rare group but are expected to increase during the next decades.

Funding Acknowledgement

Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Swedish heart lung foundation
Impact of left atrial size and strain on new atrial arrhythmias during a 5-year follow-up in adults with congenital heart disease
Authors: Nussbaumer C , Schwitz F , Elchinova E , Goulouti E , Wustmann K , Papa A , Schwerzmann M
Published: October 2022

Introduction

Atrial arrhythmias are a common and important cause of morbidity and mortality in adults with congenital heart disease (ACHD). In acquired heart disease, left atrial (LA) strain has been shown to predict supraventricular tachyarrhythmias (SVT). This study sought to investigate if LA strain is also a reliable predictor of SVT in the ACHD population.

Method

We retrospectively obtained baseline clinical and echocardiographic data, including LA function parameters and strain, in 206 ACHD patients. Only patients with sinus rhythm at baseline and 5-years follow-up were included (median age 29, IQR 22–41 years). 157 participants had a left heart defect (aortic stenosis or aortic coarctation, with or without correction) and 49 a right heart defect (Fallot physiology). Diagnosis of sustained SVT was determined from clinical reports during the follow-up period (standard 12-lead ECG, ECG Holter).

Results

During a median follow-up of 6.2 years, a new or recurrent sustained SVT occurred in 16 patients (7.8%). Patients baseline characteristics are depicted in Table 1. Patients who developed SVT were older, had larger LA dimensions and left ventricular mass, more likely diastolic dysfunction on echo, and a lower peak LA longitudinal strain (PALS). PALS was a good predictor of SVT risk in patients with left and right heart defects with an area under the receiver-operating-curve of 0.857. By Cox regression analysis, patient in the lowest quartile for PALS had a 16.7-fold higher hazard ratio of SVT (95% confidence interval, 4.7 to 59.0, p<0.001) in comparison with the top three quartiles. Overall freedom from arrhythmia after 1, 3 and 5 years of follow-up was 98.1%, 96.1% and 94.2%, respectively. The freedom from SVT as a function of PALS quartiles is shown in Figure 1.

Conclusion

PALS provides predictive information about the occurrence of SVT in the ACHD population, regardless of the type of the lesion. Including the measurement of LA strain in the follow-up of these patients may permit to better identify patients at risk of future atrial arrhythmias.

Funding Acknowledgement

Type of funding sources: Public hospital(s). Main funding source(s): Universitätsklinik für Kardiologie, Inselspital Bern
Clinical and cardiovascular magnetic resonance predictors of early and long-term clinical outcome in acute myocarditis
Authors: Bohbot Y , Garot J , Hovasse T , Unterseeh T , Di Lena C , Boukefoussa W , Tawa C , Renard C , Limouzineau I , Duhamel S , Garot P , Tribouilloy C , Sanguineti F
Published: October 2022

Background/Introduction

The natural history of acute myocarditis (AM) remains partially unknown and predictors of outcome are debated.

Purpose

We sought to assess the impact of various cardiac magnetic resonance (CMR) parameters on early and long-term prognosis in a population of patients with AM.

Methods

In a two-centre longitudinal study, we included consecutive patients with diagnosis of AM based on CMR and without haemodynamic compromise. The primary endpoint was the occurrence of an event in the acute phase (≤15 days). Secondary endpoints were the occurrence of major adverse cardiac events (MACE) and recurrence of AM during follow-up.

Results

Three hundred and eighty-eight patients were included (mean age 38.5 years, 77.3% male, mean ejection fraction [EF]:56%) of which 82% (317) presented with chest pain. CMR was performed 4±2 days after index presentation. Overall, 38 patients (9.8%) had an event at the acute phase, 41 (10.6%) presented at least one MACE during follow-up (median 7.5 years, 6.6–8.9) and 30 (7.7%) experienced a recurrence of AM. By multivariate analysis, the independent predictors of initial complications were absence of chest pain (OR [95% CI] = 0.35 [0.15–0.82]), presence of syncope/pre-syncope (OR [95% CI] = 3.56 [1.26–10.02]), lower EF (OR [95% CI] = 0.94 [0.91–0.98] per %), myocardial extent of late gadolinium enhancement (LGE) (OR [95% CI] = 1.05 [1.002–1.100] per %) and absence of edema (OR [95% CI] = 0.44 [0.19–0.97]). Only age (HR [95% CI] = 1.021 [1.001–1.041] per year) and an initial alteration of EF (HR [95% CI] = 0.94 [0.91–0.97] per %) were associated with MACE during follow-up. Factors independently associated with AM recurrence were myocarditis prior to the index episodes (HR [95% CI] = 5.74 [1.72–19.22]) and viral syndrome at the index episode (HR [95% CI] = 4.21 [1.91–9.28]).

Conclusion

In routine consecutive hemodynamically stable patients with diagnosis of AM based on CMR, absence of edema, reduced EF, and extent of LGE were associated with early adverse outcome. Only age and EF were associated with long-term events.

Funding Acknowledgement

Type of funding sources: None.
Outcome of device atrial septal defect closure in different age groups: a nationwide cohort study
Authors: Muroke V , Jalanko M , Pihkala J , Hartikainen J , Tahvanainen A , Airaksinen J , Ukkonen H , Kervinen K , Ylitalo K , Sinisalo J
Published: October 2022

Background

Catheter based ASD closure has been a mainstay therapy for secundum type ASD for over 20 years and up to 80–90% of defects are deemed eligible for catheter closure. We are starting to get long term results for the outcome of the procedure. Numerous studies have reported excellent prognosis and low complication rates in patients with TC closed defects. However, little is known about the disease burden in the long run after the closure.

Objectives

This nationwide cohort aimed to evaluate long-term outcome of transcatheter closed atrial septal defects (ASD) by evaluating 1) hospitalisations and disease burden, 2) complications and 3) echocardiographic changes.

Material and methods

We gathered every transcatheter ASD closure done in Finland during 1999–2019. Mean follow-up time was 6.8 years (SD 5.1). We used hospital discharge register to study the incidence of new onset atrial fibrillation, stoke and migraine after the closure. Closure complications and echocardiographic changes were gathered from the electronic health records.

Results

Overall transcatheter ASD closure was performed for 1000 patients (68.5% females) during the study period. ASD patients had increased risk for new-onset atrial fibrillation (RR 2.55, 95% CI: 1.94–3.36), migraine (RR 3.41, 95% CI: 2.40–4.84) and heart failure. Stroke risk was not increased during the follow-up (p=0.19). Hospitalisations were more common in the ASD group in the following 12 months after the closure (RR 2.52, 95% CI: 2.14–2.98). Adverse events occurred in 6.9% (n=69) of the patients, including 4 erosions and 10 device embolisations.

Right heart size was enlarged in 83.9% of the patients before the closure and in 24.4% after the closure. Rate of mild mitral regurgitation increased 21.2% vs. 27.6%, p=0.005) and rate of sever to moderate tricuspid regurgitation decreased (9.4% vs. 5.6%, p=0.009) during the follow-up.

Conclusion

Catheter based Atrial septal defect closure is a safe procedure with low complication rates. TC closed ASD patients had more new-onset atrial fibrillation, heart failure and migraine during the follow-up.

Funding Acknowledgement

Type of funding sources: Foundation. Main funding source(s): The Emil Aaltonen Foundation, Tampere, Finland
Percutaneous interventions in Fontan circulation
Authors: Marschall A , Alvarez-Fuente M , Garrido-Lestache E , Tellez L , Sanchez I , Toledano M , Molina I , Rivero N , Garcia-Ormazabal I , Tamariz R , Del Cerro M J
Published: October 2022

Background

In light of increased survival of patients with Fontan circulation, percutaneous interventions in these patients have become growingly significant in the recent years. Percutaneous treatments in patients with univentricular physiology can optimize the short term hemodynamics in these patients. However, information on long term outcomes is scarce.

Purpose

To present the cumulative work experience in percutaneous interventions of a tertiary center in the past 12 years in patients that have undergone total cavopulmonary deviation (TCD) and to investigate long term outcomes.

Methods

In this retrospective study, all patients with TCD that underwent follow-up in our specialized department of congenital heart disease in the past 12 years, were eligible for chart-review. Cardiac catheterization was performed by experienced interventional cardiologists, specialized in structural interventions in congenital heart disease. All catheterizations were performed under general anesthesia and mechanical ventilation. All patients underwent Fontan pressure measurements and cardiac output estimation with Fick method.

Results

A total of 80 patients, with a mean follow-up time of 24 (±7.1) years, were included in the study. Details on baseline characteristics are depicted in Table 1. Fifty-two (65%) patients underwent percutaneous intervention during follow-up, and 33% (n=17) of these patients required multiple interventions. Angioplasty with stent implantation of the Fontan conduit or the pulmonary arteries was the most common intervention (30 (58%) and 8 (15%) patients, respectively), followed by embolization of collaterals (11 (21%) patients). Patients with right systemic ventricle physiology needed more interventions and had a shorter time-to-first-intervention period. (Figure 1) Pacemaker implantation was required in a total of 15 patients, being significantly more frequent in patients with atriopulmonary connection than in patients with lateral tunnel or extracardiac conduit (11 (36%) vs 4 (11%), p<0.01). Overall long-term outcome (with regards to mortality) was good (4 (0.5%) patients). Patients that required percutaneous structural interventions showed a tendency towards a higher mortality than those not in need of intervention (4 (8%) vs 0 (0%), respectively, p=0.14).

Conclusions

During long-term follow-up of patients with Fontan circulation, almost two thirds of the patients required percutaneous intervention. Patients with right systemic ventricle physiology were in greater need of intervention. Patients that required intervention had a tendency towards a worse clinical outcome.

Funding Acknowledgement

Type of funding sources: None.
Percutaneous edge-to-edge repair for systemic tricuspid valve regurgitation in patients with congenital heart disease: the first descriptive cohort
Authors: Guerin P , Jalal Z , Cueff C , Hascoet S , Bouvaist H , Levy F , Hugues N , Ladouceur M , Malekzadeh-Milani S G , Iriart X , Silini A , Karam N , Iserin L , Le Gloan L , Thambo J B
Published: October 2022

In patients with congenital heart diseases (CHD) systemic tricuspid regurgitation (STR) is frequent.

Here, we report our experience with percutaneous edge-to-edge repair (PETER) in a series of 12 CHD patients with STR using the MitraClip system.

Twelve patients (median age = 35 years, 83% males) with severe STR (ccTGA (n=5), asTGA (n=3), SV (n=4)), at high-risk for surgical treatment, gave informed consent for treatment using PETER. The XTR MitraClip device (Abbott, Santa Clara, CA) was used. At baseline, 7/12 patients were in NYHA functional class ≥ III. Standard femoral venous access was successfully used in 10/12 patients. In SV patients, the systemic valve was accessed directly through the right atrium (n=2), after a Fontan conduit puncture (n=1), or after direct atrial surgical access (n=1). In TGA patients, either transseptal (ccTGA patients, n=4) or transbaffle (asTGA patients, n=3) puncture was performed (4).

Mitraclip devices were successfully implanted in 11/12 patients (Figure 1). One procedural complication was reported for a patient with SV in whom a septal leaflet rupture occurred following clip release, leading to severe STR; the patient died from refractory cardiac failure one week later. Peri-procedural complications included one case of left femoral vein injury and one case of atrial flutter treated by catheter ablation. After a median follow-up of 12-months (range 1–25) following device implantation, no death had occurred. The patients had significant reduction in STR (from severe to moderate in 10/11 patients) and clinically improved (10/11 patients were in NYHA class I or II).

To conclude, percutaneous edge-to-edge therapy of STR in CHD patients is feasible, safe and effective to reduce STR and SRV dilatation. This reduction is associated with a significant clinical improvement.

Funding Acknowledgement

Type of funding sources: None.
Patent foramen ovale (PFO) and migraine: role of platelet Tissue Factor expression and oxidative stress. The LEARNER study
Authors: Camera M , Brambilla M , Canzano P , Becchetti A , Conti M , Teruzzi G , Porro B , Muratori E , Tedesco C , Montorsi P , Bartorelli A , Tremoli E , Trabattoni D
Published: October 2022

Background

Migraine is a chronic neurovascular disorder with a multifactorial aetiology. A strong relationship links migraine with aura (MHA) and patent foramen ovale (PFO), with migraine regression after PFO closure. Increased platelet aggregation and oxidative stress were documented in migraineurs. Current guidelines suggest the use of aspirin or P2Y12-antagonists. To date, however, no clear mechanisms connecting MHA to PFO has been demonstrated.

Purpose

To perform a comprehensive analysis of platelet activation, inflammation and oxidative stress status in 78 aspirin-treated MHA-patients before (T0) and 6-months after (T1) PFO closure (LEARNER Study-NCT03521193-clinicaltrials.gov). Primary endpoint was migraine regression rate in relation to these parameters.

Methods

P-selectinpos-, activated-glycoproteinIIbIIIa (aGPIIbIIIa)pos-, Tissue Factor (TF)pos-, reactive oxygen species (ROS)pos-platelets, platelet-leukocyte aggregates (PLA) and microvesicles (MVs) were evaluated by flow cytometry; thrombin generation (TG) by Calibrated Automated Thrombogram (CAT) assay; oxidative stress status by mass spectrometry. Twelve aspirin-treated-healthy subjects (HS) were enrolled for comparison. To test the effect of PFO patients' plasma on platelet activation, blood from HS was plasma-depleted and replaced with a pool of plasma from PFO patients.

Results

Migraine resolution occurred in 69.7%, significant reduction in 27%, while no effect was observed in 2 patients (3.2%). Only ROSpos-platelets, and TFpos-platelets and -MVs were significantly higher at T0, sustaining a TG capacity that was associated with an altered blood GSSG/GSH (Oxidized/Reduced Glutathione) ratio. This phenotype reverted to HS levels at T1 (Fig. 1). MHA-PFO plasma, added to HS blood, mirrored the in vivo platelet activation and N-acetylcysteine blunted it. In vitro GSSG treatment of HS platelets reproduced the in vivo condition. Aspirin had little effect on the platelet prothrombotic phenotype which was effectively inhibited by a P2Y12-antagonist (Fig.2).

Conclusions

MHA-PFO patients show a platelet-associated prothrombotic phenotype, characterized by a marked thrombin generation capacity sustained by an elevated number of platelets and MVs expressing a functionally active Tissue Factor and sustained by altered oxidative stress status. This phenotype, not fully controlled by aspirin but by P2Y12-antagonism, reverted after PFO closure together with a complete migraine remission.

Funding Acknowledgement

Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Italian Ministry of Health
Development of psychopathology in adults with congenital heart disease: a 40–53 years follow-up study
Authors: Pelosi C , Kauling R M , Cuypers J A A E , Van Den Bosch A E , Bogers A J J C , Helbing W A , Roos-Hesselink J W , Legerstee J S , Utens E M W J
Published: October 2022

Background

Nowadays, more than 90% of patients with congenital heart disease (CHD) reach adulthood. However, little is known about long-term psychopathological problems in these patients.

Purpose

To investigate the development of psychological outcomes of adults with CHD operated in their childhood.

Material and methods

Our unique, single center, longitudinal cohort-study evaluates a series of consecutive patients who underwent cardiac surgery in childhood between 1968 and 1980 every 10 years. Standardized questionnaires were used to measure psychopathology at 4 follow-up time points (1991, 2001, 2011 and 2021) and outcomes were compared with normative data. Results in the current evaluation were corrected for the Covid-19 stringency index.

Results

At the current (4th) evaluation in 2021, 204 patients (46% female, age: 49.9±5.2) participated. Female patients with CHD reported significantly more internalizing problems than the normative group. More specifically, they reported significantly more somatic complaints. Proxy-reports showed significantly less externalizing problems for males. No significant difference was found between simple and moderate/complex CHD in terms of psychopathology. Correction for Covid-19 pandemic stringency showed no significant effect.

Over time, the percentage of patients scoring in the psychopathological range decreased from the first (1991, 25.4%) to the third follow-up (2011, 1.8%). However, the current study showed a significant increase (9.6%) of the psychopathology levels in comparison with 2011. (figure1)

Conclusions

Compared to normative data, female CHD patients reported significantly more internalizing problems, particularly more somatic complaints. Initially, the level of psychopathology decreased over time. However, in the last decade, there was a significant increase in psychopathology, not explained by the Covid-19 pandemic, warranting continuous attention to the psychological health of adults with CHD.

Funding Acknowledgement

Type of funding sources: Foundation. Main funding source(s): Thorax foundation
Extracellular volume fraction by computed tomography predicts long-term prognosis among patients with cardiac amyloidosis
Authors: Gama F , Rosmini S , Bandula S , Patel K P , Thornton G D , Bennett J B , Wechelakar A , Gillmore J D , Whelan C , Lachmann H , Taylor S , Fontana M , Moon J , Hawkins P N , Treibel T
Published: October 2022

Objective

This study sought to investigate the association of extracellular volume fraction by computed tomography (ECVCT), myocardial remodeling and mortality in patients with systemic amyloidosis.

Background

Light chain (AL) and transthyretin (ATTR) amyloid fibrils are deposited in the extracellular space of the myocardium, resulting in heart failure and premature mortality. Extracellular expansion can be quantified by CT, offering a rapid and cost-effective alternative to cardiovascular magnetic resonance (CMR), especially among patients with cardiac devices or on renal dialysis.

Methods

Patients with confirmed systemic amyloidosis and varying degrees of cardiac involvement underwent ECG-gated cardiac CT. ECVCT was analysed in the inter-ventricular septum. All patients also underwent clinical assessment, ECG, echocardiography, serum amyloid protein component (SAP) and/or technetium-99m (99mTc) 3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy. ECVCT was compared across different extents of cardiac infiltration (ATTR Perugini Grade / AL Mayo Class) and evaluated for its association with myocardial remodelling and all-cause mortality.

Results

72 patients were studied (AL n=35, ATTR n=37; age 67 (59–76) years, 71% males). Mean septal ECVCT was 42.7±13.1% and 55.8±10.9% in AL and ATTR, respectively, and correlated with indexed left ventricular (LV) mass (r=0.426, p<0.001), LV ejection fraction [LVEF, (r=0.460, p<0.001)], NT-proBNP (r=0.563, p<0.001) and hsTnT (r=0.546, p=0.02). ECVCT increased with cardiac amyloid involvement in both AL and ATTR (Figure 1). Over a mean follow-up of 5.3±2.4 years, 40 deaths occurred (AL 14 [35%]; ATTR 26 [65%]). ECVCT was independently associated with all-cause mortality in ATTR (not AL) after adjustment for age and IV septal wall thickness (HR: 1.046, 95% CI: 1.003–1.090, p=0.037).

Conclusion

Cardiac amyloid burden quantified by ECVCT is associated with adverse cardiac remodelling as well as all-cause mortality among ATTR amyloid patients. ECVCT may address the need for better identification and risk stratification of amyloid patients, using a widely-accessible imaging modality (Figure 2).

Funding Acknowledgement

Type of funding sources: None.
Predictors of of arrythmia in adult patients with Ebstein anomaly
Authors: Wals Rodriguez A J , Gallego P , Pijuan Domenech A , Rodriguez Puras M J , Miranda Barrio B , Gordon Ramirez B , Ladron Abia R , Gonzalez Fernandez V , Dos Subira L
Published: October 2022

Objectives

The objective of this study was to evaluate the incidence and risk factors for arrhyhthmias in adult patients with Ebstein anomaly (EA).

Methods

Retrospective observational study of a multicenter cohort of 123 adult patients with EA. Demographic, clinical and echocardiography data at their first visit at the Adult Congenital Heart Disease Unit were collected. Free-event survival was assessed with Kaplan Meier curves and differences between groups using the log-rank test. Predictors of arrythmia were assessed using Cox regression analysis.

Results

Median follow up (FU) was 6 (IQ 2–12) years and the median age at the end of FU was 47 (IQ 33–60) years. 61.8% (n=76) were women and 68 patients (55%) had associated atrial septal defects. A total of 43 patients (34%) were repaired and 11 (8.9%) required reoperation. During FU 40 patients (32.5%) had supraventricular arrhythmias (SA), mainly atrial flutter (n=19; 15.6%) and atrial fibrillation (n=14; 11.5%); 12 patients (9.8%) developed high-grade atrio-ventricular block (AVB). In the multivariate analysis, the baseline variables associated with the appearance of SA were history of cyanosis (HR: 2.269 (1.104–4.666); p=0.026) and NYHA functional class ≥ II at baseline (HR: 2.620 (CI: 1.062–6.465); p=0.037). For high-grade AVB, the associated variable was tricuspid valve implant during FU (HR: 7.993 (CI: 1.396–45.770); p=0.020).

Conclusions

Arrhythmias, particularly SA, are a common complication in adult patients with EA. Patients with a NYHA functional class ≥ II at baseline and those exposed to cyanosis are more prone to develop SA during FU, whereas patients requiring tricuspid valve implant are at increased risk of developing high-degree AVB.

Funding Acknowledgement

Type of funding sources: Public hospital(s). Main funding source(s): Hospital Vall d'HebronHospital Univertsitario Virgen del Rocío
Predictors of long term tricuspid regurgitation after pulmonary valve replacement: a single center study on the cohort of significant preoperative tricuspid regurgitation
Authors: Carbonell Prat B , Dos L , Gordon B , Ligero C , Gonzalez V , Vilte G , Pijuan A , Miranda B , Ferre M , Lorente M , Guedes P , Ferreira I , Castro M A
Published: October 2022

Background

Almost one-third of repaired tetralogy of Fallot (TOF) and pulmonary stenosis (PS) patients referred for pulmonary valve replacement (PVR) have at least moderate tricuspid regurgitation (TR). Predictors of long term TR in this population are not well defined and the role of Tricuspid Annuloplasty (TA) in addition to PVR in this setting is controversial.

Purpose

Patients with preoperative severe TR or moderate TR with dilated tricuspid annulus (>40mm) undergo TA at the time of PVR as per protocol in our instititution. The aim of this observational study was to evaluate the long term results of this approach and to identify predictors of significant TR at the end of follow-up.

Material and methods

We retrospectively reviewed 35 repaired TOF and PS patients who underwent surgical PVR with (n=17, group 1) or without (n=18, group 2) TA in a single tertiary hospital between June-2009 and June-2021. Pre- and postoperative echocardiographic and cardiac magnetic resonance data were assessed for TR severity and right ventricle size and systolic function. Baseline and clinical follow up data were collected. Uni- and multivariable tests were used to analyze predictors of at least moderate TR after PVR.

Results

Mean age at PVR and baseline image parameters were comparable between groups, except for TR grade (p 0.006), tricuspid annulus size (p 0.018) and right ventricle outflow tract diameter (p 0.04) which were greater in group 1. TA did not confer and increased risk of surgical and peroperative complications. Intensive care unit (p 0.94) and total hospital admission days (p 0.59) did not differ among groups. At one year, TR grade significantly improved regardless of TA. At the end of follow-up (5.29±2.9 years), the degree of TR increased in both groups with no differences between groups (p 0.38) (see table 1). In univariable analysis, two independent predictors of at least moderate TR at end of follow-up were identified; mixed TR mechanism (OR 12.5, p 0.006) and severe preoperative TR grade (OR 10.21, p 0.003). Mixed TR mechanism (OR 15.78, p 0.01) and a right ventricle ejection fraction below 45% (OR 1.47, p 0.01) were identified as risk factors of significant TR in multivariable analysis (see Table 2).

Conclusions

TA seems a reasonable approach at the time of PVR in patients with severe TR or moderate TR with dilated annulus. Mixed TR mechanism and a worse right ventricular function have been identified as independent predictors factors of long term significant postoperative TR in our study cohort.

Funding Acknowledgement

Type of funding sources: None.
Impact of coronavirus disease 2019 on adult patients with congenital heart disease
Authors: Cardoso I , Branco T , Constante A , Viegas J , Grazina A , Teixeira B , Jacinto S , Castelo A , Rito T , Martins D , Ferreira R , Sousa L
Published: October 2022

Background

COVID-19 is responsible for a worldwide pandemic, causing more than 18,000 deaths to date in Portugal. Data already exists regarding the increased risk of adverse events in patients with cardiovascular diseases, however the impact of SARS-CoV-2 infection in patients (P) with congenital heart disease (CHD) is still under investigation.

Purpose

To study the impact of COVID-19 in a adult patients with CHD

Methods

Adult patients seen at the CHD outpatient's clinic at a tertiary centre, who became infected with SARS-CoV-2 infection up to December 2021 were included. Assessment of patients' symptoms, need for hospitalization and admission in an intensive care unit was assessed based on medical records.

Results

We identified seventy-nine patients (pts) with COVID-19 infection. Symptoms were present in 67 (84%). The median age was 44 (15) years, 52% were females. Eight P (10%) had complex cyanotic disease; seven Tetralogy of Fallot; five (6%) transposition of great arteries; eight (10%) right ventricle obstacle; two (3%) atrioventricular canal defect; sixteen (20%) atrial septal defect; nine (11%) ventricular septal defect; eight (10%) aortic coarctation; two (3%) had Eisenmenger syndrome. 49% of P had previous surgery or percutaneous procedure. 63% of P were at New York Heart Association (NYHA) class of I and 30% at NYHA II. Mild symptoms were reported by 56 P (71%). Ten adults (7,9%) experienced moderate symptoms (dyspnea and hypoxia) that led to hospitalization for oxygen therapy, none required mechanical ventilation. One death was reported in an 83-year-old patient with non-corrected interventricular communication and compromised biventricular function. There was a significant association between the gravity of CHD and hospitalizations (p=0.02).

Conclusion

Our pts had mainly mild to moderate symptoms and did not appear to have a disproportionately negative outcome; the need for hospitalization was more frequent in patients with higher CHD gravity. These findings are in line with the emerging data regarding COVID-19 in CHD P, and may be in part explained by the patient's young age and functional status.

Funding Acknowledgement

Type of funding sources: None.
Human versus machine: does artificial intelligence add value to identification of hypertrophic cardiomyopathy in pediatric patients?
Authors: Guerrier K , Gunturkun F , Wetzel G , Akbilgic O , Davis R , Towbin J
Published: October 2022

Introduction

An abnormal increase in left ventricular thickness is a hallmark of hypertrophic cardiomyopathy (HCM). Although standard measures of left ventricular voltage abnormalities on electrocardiograms (ECGs) have high false positive rates and poor correlation to left ventricular thickness, ECGs continue to be part of most screening programs. We developed a machine learning model for HCM classification from ECG in a pediatric cohort and compared its efficacy to that of clinician specialists.

Purpose

To compare clinician-based predictions of HCM to a machine learning model trained to classify pediatric patients with HCM utilizing 12-lead ECG.

Methods

ECGs from patients <19 years with HCM, including those with HCM gene mutations, were compared to those from age- and sex-matched controls with normal heart structure and function on echocardiogram. Patients with a known history of primary causes of left ventricular hypertrophy such as aortic stenosis or glycogen storage disease were excluded. A cascaded convolutional neural network was developed combining a residual neural network with a 2-layer dense neural network with 10-fold cross validation. The performance of the machine learning based HCM classification was compared to that of two independent clinicians.

Results

Analytic sample included data from 82 patients with clinical HCM compared to 91 healthy control subjects. The machine model AUC was 0.89 (0.84–0.94). Clinician inter-rater reliability was 0.8. The clinicians had a higher specificity (97% vs 82%) but lower sensitivity (50% vs 78%) than the machine learning model. Compared to clinician-classification, the positive predictive value was lower in the machine model (82% vs 93%).

Conclusion

In this preliminary study, machine learning classification of HCM utilizing 12-lead ECG had greater sensitivity than that of clinician interpretation. Machine learning may play a role in screening and diagnosis, including in subjects with normal-appearing ECG. Evaluation of its utility in a larger cohort is needed.

Funding Acknowledgement

Type of funding sources: Public hospital(s). Main funding source(s): Le Bonheur Children's Hospital
The association between age, troponin level, and mortality in patients hospitalised with acute pulmonary embolism (NIHR Health Informatics Collaborative TROP-PE study)
Authors: Kaura A , Goswami S , Mulla A , Glampson B , Davies J , Woods K , Shah A M , Kharbanda R , Patel R S , Perera D , Channon K M , Quint J , Mayet J
Published: October 2022

Background

A positive cardiac troponin (cTn) is an independent predictor of short-term mortality in individuals presenting with acute pulmonary embolism (PE). However, there is limited evidence regarding the impact age has on the association between cTn levels and mortality in patients with PE.

Purpose

The aim of our study was to investigate the relationship between cTn level, age, and all-cause mortality, in hospitalised patients diagnosed with an acute PE.

Methods

A retrospective cohort study using the National Institute for Health Research Health Informatics Collaborative Cardiovascular dataset of all consecutive patients who had a troponin measured at five hospitals between 2010 and 2017. Patients admitted to hospital with a primary diagnosis of PE with at least one cTn measurement were included. We modelled the relation between peak troponin level and all-cause mortality using multivariable adjusted restricted cubic spline Cox regression analyses. Effect estimates were adjusted for age, gender, high-sensitivity troponin assay, C-reactive protein, haemoglobin, platelet count, white cell count, creatinine, sodium, potassium, diabetes, hypertension, hypercholesterolaemia, acute coronary syndrome, atrial fibrillation, heart failure, acute kidney injury, chronic kidney disease, obstructive lung disease, inflammatory disorders, pneumonia and malignancy. The peak cTn level (highest level measured), standardised to the upper limit of normal (ULN), was used for all analyses.

Results

1,477 patients with at least one cTn measurement and a diagnosis of acute PE were included. During a median follow-up of 34.8 months, there were 290 (19.6%) deaths. Elevated cTn (>1xULN) was associated with a hazard ratio (HR) of 3.29 (95% confidence interval [CI] 1.95–5.53) for 30-day mortality and 2.12 (95% CI 1.63–2.75) for 3-year mortality. Higher cTn levels were progressively associated with a higher mortality risk, reaching a maximum HR of 2.59 (95% CI 1.64–4.09) at 141xULN (Figure 1). Younger patients (<55 years), compared with those aged over 55, had the highest 3-year HR associated with a positive cTn of 2.94 (95% CI 1.48–5.82) despite having the lowest troponin levels (mean 7.01xULN) on admission (Figure 2).

Conclusion

Elevated cTn, at all ages, is associated with an increased mortality risk in patients presenting with PE, with increasing cTn levels conferring a progressively worse long-term prognosis. Elevated cTn, no matter how small, needs to be taken seriously, particularly in young patients with an acute PE.

Funding Acknowledgement

Type of funding sources: Public grant(s) – National budget only. Main funding source(s): 1) NIHR Imperial Biomedical Research Centre, as part of the NIHR Health Informatics Collaborative, and 2) British Heart Foundation
Paediatric pulmonary arterial hypertension following congenital heart defect repair: enhanced risk stratification and outcomes in a national cohort
Authors: Constantine A , Dimopoulos K , Condliffe R , Clift P , Jansen K , Dhillon R , Chaplin G , Muthurangu V , Moledina S
Published: October 2022

Introduction

Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD). PAH following CHD repair (repaired PAH-CHD) is an emerging population with increased morbidity and mortality [1–3]. Risk scores that are widely used in the adult PAH population have not been validated and may not be applicable in young children with repaired PAH-CHD.

Purpose

To characterize the 20-year cohort of the UK National Paediatric PH Service, focusing on peri-operative characteristics and outcomes of children with repaired PAH-CHD, and develop a tailored risk stratification tool.

Methods

We included consecutive children presenting to our specialist service between 2001 and 2021 with a diagnosis of repaired PAH-CHD. Patients with univentricular physiology, segmental pulmonary hypertension (PH), and PH primarily due to abnormal development of the pulmonary vasculature were excluded. Univariable Cox regression analysis was performed in children with PAH present ≥3 months after CHD repair, with an outcome of death or transplantation. Time-dependent ROC analysis was used to evaluate the risk model.

Results

Overall, 178 patients were included (age 3.2 [1.3–7.9] years, 58.4% female). Most children (73.0%) were referred following CHD repair. Complex CHD was present in 61.2%, and 48.9% had combined pre- and post-tricuspid shunts. Down syndrome was present in 33.1%. At the first post-operative PH assessment, 53.1% of patients had symptoms, mainly breathlessness. On echocardiographic evaluation, 30.9% had moderate-severe right ventricular dilatation and 23.7% had right ventricular systolic impairment. Over a median follow-up of 6.0 [2.4–11.0] years, 30 (19.2%) patients died and 5 (3.2%) patients underwent lung transplantation. Transpant-free survival at 1, 5, and 10 years was 94.7% (95% CI: 91.2–98.3%), 85.9% (95% CI: 80.3–91.9%), and 80.1% (95% CI: 73.1–87.7%), respectively. Cox analysis identified several clinical variables associated with death or transplantation, of which the following variables assessed post-repair were used to develop the risk score: absence of pre-operative PH, breathlessness, right ventricular dysfunction, and pulmonary vascular resistance index >14 WU m2 or lesion at high risk of causing PH (Figure 1). This novel, simple risk score performed well (AUC >80% at 1, 3, 5 and 10 years) and was well-calibrated in our cohort (Figure 2, D'Agostino-Nam p=0.76).

Conclusions

In this national cohort of children with repaired PAH-CHD, mortality is significant. This novel, simple risk score performed well in our population and could be used in clinical practice at the time of post-operative assessment to predict outcome and direct management but requires further validation.

Funding Acknowledgement

Type of funding sources: None.
Increased risk of long-term mortality following pulmonary embolism in Denmark, 2000–2020: a nationwide trend study
Authors: Fredslund Madsen S , Moelager Christensen D , Strange J E , Nourhavesh N , Kumler T , Gislason G , Lamberts M , Sindet-Pedersen C
Published: October 2022

Background

New diagnostics and treatment regimens have been introduced in the last 20 years which may have influenced the prognosis of pulmonary embolism (PE). However, there is a lack of studies investigating temporal trend in short- and long-term mortality following PE in different subgroups.

Purpose

We aimed to investigate temporal trends in short- and long-term mortality following PE according to age, sex, and cancer status.

Method

This study was based on data from Danish national registries. All patients with a first-time inpatient hospital diagnosis of PE between the 1st of January 2000 through 3rd of December 2020 were included in the study. Mortality was calculated as the proportion of patients who died within 30 days and 31–365 days, respectively, and were presented with 95% confidence intervals (CI), and additionally stratified into categories of age groups (18–39, 40–59, 60–79, and 80–100 years), sex (male/female) and cancer status (no cancer/cancer). Cancer was defined as a cancer diagnosis registered within 6 months prior to the PE diagnosis.

Results

In total 62,750 patients had a first-time PE during the study period. The mean age of the study population was 68.3 years (sd), and 32,617 (51.9%) were women and 18,719 (29.83%) of patients had cancer. The 30-day mortality for age groups, sex, and cancer status decreased during the study period 2000–2020 (Figure 1A). The 31–365-day mortality for age group 40–59 years increased from 5.7% [95% CI: 3.7–8.5] in 2000 to 9.5% [95% CI: 7.5–11.9] in 2019. Age group 80–100 years increased from 18.5% [95% CI: 14.8–22.8] in 2000 to 23.5% [95% CI: 21.0–26.2] in 2019. No temporal differences were observed for age groups 18–39 and 60–79 years. Mortality for cancer-associated PE increased from 24.8% [95% CI: 19.8–30.6] in 2000 to 29.9% [95% CI: 27.4–32.4] in 2019 (Figure 1B).

Conclusion

Despite overall mortality following PE has improved, our data suggest that short-term mortality following pulmonary embolism has improved considerably while long-term mortality were increased. Future studies should assess whether this phenomenon is due to a different patient risk profile (e.g. cancer) or lack of longer term care (e.g. rehabilitation program, withdrawal of antithrombotic treatment).

Funding Acknowledgement

Type of funding sources: None.
Reappraisal of echocardiographic algorithm in predicting the pulmonary hypertension redefined by updated pulmonary artery mean pressure treshold
Authors: Tanyeri S , Tokgoz H C , Karagoz A , Akbal O Y , Keskin B , Kultursay B , Hakgor A , Kulahcioglu Ş , Ceneli D , Tosun A , Efe S C , Bayram Z , Tanboga I H , Ozdemir N , Kaymaz C
Published: October 2022

Background

Although an adopted echocardiography algorithm based on tricuspid regurgitation jet peak velocity (TRVmax) and suggestive findings (SF) for pulmonary hypertension (PH) has been utilized in the non-invasive prediction of PH probability, reliability of this approach for updated hemodynamic definition of PH remains to be determined.

Aims

In this study, first time, we aimed to evaluate the TRVmax and SF in predicting the probability of PH as defined by mean pulmonary arterial pressure (mPAP) >20 mm Hg and mPAP >25 mm Hg, respectively.

Methods

Our study group comprised of the retrospectively evaluated 1300 patients (age 53.1±18.8 years, female 62.1%) who underwent right heart catheterisation (RHC) with different indications between 2006 and 2018. All echocardigraphic and RHC assessments were performed in accordance with European Society of Cardiology (ESC)/European Respiratory Society (ERS) 2015 PH Guidelines.

Results

Although TRVmax showed a significant relation with mPAP in both definitions, SF offered significant contribution for only in predicting mPAP >25 mm Hg, but not for mPAP >20 mm Hg. In predicting the mPAP >20 mm Hg TR Vmax and SF showed odds ratio (OR) of 2.57 (1.59–4.14, p<0.001) and OR of 1.25 (0.86–1.82, p=0.16), respectively (Table 1). In predicting the mPAP >25 mm Hg, TR Vmax and SF showed OR of 2.33 (1.80–3.04, p<0.001) and OR of 1.54 (1.15–2.08, p<0.001), respectively (Table 1). The TRVmax >2.8 m/sec and TRVmax >3.4 m/sec were associated with 70% and 84% probability of mPAP >20 mm Hg, and 60% and 76% probability of mPAP >25 mm Hg, respectively (Figure 1).

Conclusions

In contrast to those in predicting the mPAP >25 mm Hg, SF did not provide a significant contribution to probability of mPAP >20 mm Hg predicted by TRVmax solely. The impact of the novel mPAP treshold on echocardiographic prediction of PH remains to be clarifed by future studies.

Funding Acknowledgement

Type of funding sources: None.
Differences in the respiratory swings in COPD and ILD candidates for lung transplantation: a critical concern interpreting central pulmonary pressures
Authors: Grignola Rial J , Calabuig A , Trujillo P , Bravo C , Casas G , Azpiroz F , Lopez Messeguer M , Domingo E
Published: October 2022

Background

In different clinical scenarios (i.e., obesity, COPD, exercise, mechanical ventilation), the swings in intrathoracic pressure are much larger, and end-expiratory (Pee) pressure can be significantly greater than atmospheric pressure. In these scenarios, the intravascular (Piv) pulmonary pressure can overestimate the true transmural (tm) value and it is recommended to read the average of Piv over a few respiratory cycles (Pmrc) [1–3].

Purpose

To analyze the respiratory swings and the effect of esophageal pressure (PES) (as a surrogate of intrathoracic pressure) on the reading of Piv tracings during the RHC at rest in COPD and interstitial lung disease (ILD) candidates for lung transplantation (LTx).

Methods

Thirty-one COPD (15) and ILD (16) candidates for LTx underwent RHC. End-expiratory and mean respiratory cycle measurements were obtained. The respiratory swing was estimated as the difference between maximum-minimum values of Piv. Ten patients (5 COPD/5 ILD) underwent simultaneous RHC and PES (Micro-balloon Esophageal Catheter, LATITUDE) to assess the Ptm (Piv − PES) [4].

Results

Both demographic (11F/20M, 60±7 yrs, 25±4 kg/m2) and hemodynamic data (mPAP 24±9 mmHg, pulmonary arterial occlusion pressure [PAOP] 8.6±4 mmHg, right atrial pressure [RAP] 5.2±3.9 mmHg, pulmonary vascular resistance 3.5±2.6 Wu) did not show significant differences between ILD and COPD except the cardiac index (ILD: 2.8±0.8 vs. COPD: 2.4±0.3 L/min/m2). Intravascular RAPee and PAOPee were higher than mrc values in both groups (Fig 1). However, transmural RAPee and PAOPee were similar to and correlated with (r=0.62 and 0.69, respectively; p<0.05) transmural Pmrc values. PESee values were positive in COPD and ILD (3.0±2.2 vs. 3.3±2.0 mmHg, NS). All ILD had negative PESmrc values and were lower than COPD patients (−1.76±1.7 vs. 0.78±1.6 mmHg, p<0.05). ILD PES swings were higher than COPD (10.9±3.7 vs. 8.3±1.4 mmHg), although it did not reach statistical significance (p=0.08). The lower forced vital capacity (%), the more negative the PESmrc (Fig 2A). ILD pts showed higher transmural to intravascular Pmrc (p<0.05) (Fig 2B).

Conclusion

End-expiratory intravascular RAP and PAOP overestimates the mean respiratory cycle pressures in COPD and ILD candidates for LTx. Averaging pulmonary vascular pressure tracings over the respiratory cycle would be accurate in COPD but could underestimate transmural values in ILD candidates for LTx. The reading of mean respiratory cycle pressure could not be enough to correct the pulmonary pressures measurement error associated with the presence of large swings of intrathoracic pressure.

Funding Acknowledgement

Type of funding sources: None.