bluuebunny/crossref_metadata_2025 huggingface dataset:
Prognostic implications of biventricular uptake of bone tracers at planar scintigraphy in transthyretin cardiac amyloidosis
Authors: Porcari A , Pagura L , Canepa M , Biagini E , Cappelli F , Tini G , Dore F , Longhi S , Sciagra' R , Fontana M , Gillmore J , Rapezzi C , Merlo M , Sinagra G
Published: October 2022

Background

The prognostic role of bone tracer uptake in transthyretin cardiac amyloidosis (ATTR-CA) is controversial. A further characterization of cardiac retention measured by Perugini scale with differentiation between biventricular (BiV) and isolated left ventricle (LV) uptake has never been attempted previously.

Purpose

The study investigated the potential prognostic significance of BiV uptake in ATTR-CA.

Methods

In this multicentre, observational study, we analysed data of ATTR-CA patients who underwent bone tracer scintigraphy with acquisition of both planar and single photon emission computed tomography (SPECT) imaging. Cardiac uptake was defined according to the Perugini visual scale. Planar BiV uptake was defined according to right ventricle (RV) uptake: 0= absent, 1= < bone, 2= equal to bone, and 3= > bone and confirmed by SPECT imaging. The primary outcome was a composite of cardiac death or hospitalization for heart failure. The secondary outcome was all-cause mortality.

Results

All 124 ATTR-CA patients enrolled had LV and RV free wall uptake on SPECT images. Of them, 93 (75%) had BiV uptake visible on planar scintigraphy. BiV uptake was found in 14%, 70%, and 92% of Perugini grade 1, 2 and 3 respectively. Compared to those with isolated LV uptake, patients with BiV uptake were older (81 vs 77 years, p=0.006) and more frequently in NYHA≥3 (32% vs 10%, p=0.018). During a median follow-up of 21 months, BiV uptake was associated with a greater occurrence of the primary outcome compared to isolated LV uptake (40% vs 19%, p=0.021), whereas the Perugini scale was not (p=0.2) (Figure 1). At multivariable analysis, NYHA class ≥3 (hazard ratio [HR] 8.1, p=0.007), eGFR <60 ml/min (HR 2.1, p=0.025) and higher degree of RV uptake (HR 1.69, p=0.007) emerged as independent prognostic parameters. In an external cohort of 463 ATTR-CA patients with a median follow-up of 30 months, planar BiV uptake was independently associated with all-cause mortality, with an incremental risk in higher grades of RV uptake (p<0.001) (Figure 1).

Conclusions

The presence of BiV uptake at planar scintigraphy identified ATTR-CA patients with worse cardiovascular and global outcomes (Figure 2), potentially serving as a novel prognostic marker.

Funding Acknowledgement

Type of funding sources: None.
Quantification of aortic valve calcification in contrast enhanced computer tomography
Authors: Laohachewin D , Hein M , Ruile P , Jander N , Neumann F J , Minners J
Published: October 2022

Introduction

Aortic valve calcification (AVC) is linked to higher gradients and worse prognosis in aortic valve stenosis. Current guidelines recommend calcium scoring of the aortic valve as additional factor for decision making in low-flow, low-gradient aortic stenosis. Calcium Scoring is performed by native computed tomography (CT). For planning of interventional valve replacement (TAVR) a second scan with contrast agent (CECT) is needed at the cost of additional radiation exposure and work up time. The aim of this study is to assess and validate an estimate conversion factor of aortic valve quantification by using CECT without native imaging.

Methods

The Agatston score was measured on native CT in 45 patients and used as a reference. In contrast enhanced CT, mean aortic attenuation values in Hounsfield units (HU) were determined by placement of a region of interest in the ascending aorta with a standard deviation of 4xHU for calcification threshold. AVC was segmented semi-automatically. A conversion formula was created by plotting of native Agatston score versus aortic valve calcification segmented on contrast enhanced CT and linear regression. Validation of calculated conversion formula was established by Intra-class coefficient (ICC) and Bland Altman analysis.

Results

The linear regression model yielded an Agatston score conversion formula of 691 + 1.83 × AVCCECT. Validation of the formula in a validation cohort (n=20) showed high agreement (r2=0.802, ICC 0.915 (CI 95%: 0.786–0.966) p<0.001, p=0.055).

Conclusion

Approximation by our calculated conversion factor from contrast enhanced CT has shown excellent reliability to determine aortic valve calcification. Further studies are needed to extend this finding to larger cohorts and diverse clinical environments.

Funding Acknowledgement

Type of funding sources: None.
Neutrophile-lymphocyte ratio and outcome in takotsubo syndrome
Authors: Zweiker D , Pogran E , Gargiulo L , El-Razek A , Vosko I , Rechberger S , Bugger H , Christ G , Bonderman D , Kunschitz E , Zirlik A , Bauer A , Metzler B , Steinwender C , Huber K
Published: October 2022

Introduction

Takotsubo syndrome (TTS) is an important form of acute heart failure with significant risk of acute complications and death. In this analysis we sought to identify predictors for in-hospital clinical outcome in TTS patients by concentrating on routine laboratory parameters at admission.

Methods

In this analysis from the Austrian national TTS registry, univariable and multivariable analyses were performed to identify significant predictors for severe in-hospital complications requiring immediate invasive treatment or leading to irreversible damage, such as cardiogenic shock, intubation, stroke, arrhythmias and death. Furthermore, the influence of identified predictors with long-term survival was evaluated.

Results

A total of 338 patients (median age 72 years, 86.9% female) from 6 centres were included. Severe in-hospital complications occurred in 14.5% of patients, including cardiogenic shock (9.8%), death (3.3%) and intubation (1.2%), respectively. Patients with complications during the hospital stay had more prevalent chronic kidney disease (CKD), were less often previous smokers and TTS was less often preceded by an emotional trigger. C-reactive protein and neutrophile lymphocyte ratio (NLR) was higher in patients with complications, and midventricular ballooning and reduced left ventricular ejection fraction (LVEF) were more prevalent. In multivariable analysis, high NLR (OR 1.04 [95% CI 1.02–1.07], p=0.009) and low LVEF (OR 0.92 [0.90–0.95] per %, p<0.001) remained significant predictors for severe in-hospital complications. Both the highest NLR tercile and the lowest LVEF tercile were associated with significantly reduced 5-year survival.

Discussion

Low LVEF and high NLR at admission were independently associated with increased in-hospital complications and reduced long-term survival in TTS patients. NLR is a new easy-to-measure tool to predict worse short and long-term outcome after TTS.

Funding Acknowledgement

Type of funding sources: None.
Molecular and haemodynamic interplay in bicuspid aortic valve aortopathy: segmental differences across the aortic circumference
Authors: Sophocleous F , De Garate E , Bigotti M G , Anwar M , Chamorro-Jorganes A , Rajakaruna C , Bucciarelli-Ducci C , Caputo M , Emanueli C , Biglino G
Published: October 2022

Background

Bicuspid aortic valve (BAV) patients develop ascending aortic (AAo) dilation. The pathogenesis of BAV-aortopathy (genetic vs. hemodynamic) remains unclear.

Purpose

To identify regional changes around the AAo wall in BAV patients with aortopathy integrating clinical imaging and molecular data.

Methods

BAV patients with aortopathy (n=20) were recruited prospectively to surgically collect aortic tissue and perform four-dimensional cardiac magnetic resonance (CMR) scans. Molecular markers were measured analysing the tissue biopsies (n=15) and wall shear stress (WSS) across the AAo circumference was calculated from the 4D CMR data (n=11 patients, and n=7 additional healthy volunteers for comparison). Dilated (anterior/right) vs. non-dilated (posterior/left) circumferential segments were profiled for whole genomic microRNAs (Next Generation RNA-Sequencing, miRCURY LNA PCR), proteins content (Tandem Mass Spectrometry) and elastin fragmentation and degeneration (histomorphometric analysis). Picture 1 summarises the study approach (including a map of the AAo divided in the following segments: A=anterior, AR=anterior-right, PR=posterior-right, P=posterior, PL=posterior-left, AL=anterior-left).

Results

Integrated bioinformatic analyses of RNA-sequencing and proteomic datasets identified 5 microRNAs (miR-128-3p, miR-210-3p, miR-150-5p, miR-199b-5p, and miR-21-5p) differentially expressed across the AAo circumference. Among them, three miRNAs (miR-128-3p, miR-150-5p, and miR-199b-5p) were predicted to have an effect on eight common target genes, whose expression was dysregulated according to proteomic analyses and involved in vascular endothelial growth factor signalling, hippo signaling and arachidonic acid pathway. Decreased elastic fibre levels and elastic layer thickness were observed in the dilated segments in the histomorphometric analysis. Increased WSS and flow-velocity, helical streamlines and asymmetrical flow were observed at the anterior/right wall in asymmetrically and symmetrically dilated patients (the latter exhibiting higher WSS), compared to healthy volunteers.

Conclusion

This study has newly revealed concomitant expressional dysregulation of miRNAs, proteins, and elastic fibres on the anterior/right wall in dilated BAV patients, corresponding to regions of elevated WSS, contributing to advance the understanding of the interplay of genetic and hemodynamic factors underpinning BAV aortopathy.

Funding Acknowledgement

Type of funding sources: Foundation. Main funding source(s): British Heart Foundation and Above & Beyond
Ischemic stroke in patients with congenital heart disease and atrial fibrillation
Authors: Holmgren A , Dellborg M , Mandalenakis Z , Giang K W , Eriksson P , Fedchenko M
Published: October 2022

Background

Atrial fibrillation (AF) is one of the most common arrhythmias in patients with congenital heart disease (CHD). The risk of developing AF is more than 20 times higher in patients with CHD compared with non-CHD controls. However, while it is known that young non-CHD patients with AF have a low risk for ischemic stroke (IS), little is known about the risk for IS young CHD patients with AF.

Purpose

To investigate the risk of developing IS in younger patients with CHD and AF compared to non-CHD control patients with AF.

Method

Using data from the National Swedish Patient Register and Cause of Death register, all CHD and born in Sweden between 1970 and 2017 with history of AF were identified. The Swedish total population register was used to identify age and sex matched controls. Among the matched controls, non-CHD patients AF were identified and used as reference group. CHD and non-CHD patients were followed from onset of AF until index IS, death or end of study (31st December 2017). Cox proportional hazard regression model with hazard ratio (HR) and 95% confidence interval (CI) was used to estimate the risk of IS in CHD patients with AF compared with non-CHD controls with AF.

Results

A total of 951 CHD with AF and 606 non-CHD controls with AF were identified. With ages ranging from 0 to 47 years, the median age was 25.6 and 30.7 years for CHD and non-CHD respectively. In patients with CHD and AF, 2.9% of patients (n=28) developed IS during follow up (mean follow up 34 years, SD ± 11.2), compared 0.5% (n=3) in non-CHD controls with AF (mean follow up 37 years, SD ± 9.5 years). The unadjusted HR for IS in CHD patients with AF compared with non-CHD controls with AF was 4.61 (95% CI1.39–15.25). When adjusted for age, sex, hypertension and heart failure, the risk of IS was more than five times higher (HR 5.16, 95% CI 1.52–17.46) in CHD patients compared with non-CHD patients. The adjusted HR for developing IS in non-complex CHD with AF compared with matched controls was 4.56 (95% CI 1.00–20.79). For patients born 1970–1989 the HR of IS was 7.35 (95% CI 1.70–31.75).

Conclusion

In this nationwide, register-based cohort study, the absolute number of IS among younger CHD patients with AF was relatively low. However, the risk for developing IS was more than 5 times higher compared to non-CHD patients with AF; highlighting the need of stroke score models in younger population, particularly to CHD patients.

Funding Acknowledgement

Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Swedish state under an agreement between the Swedish government and city Councils
Long term outcomes of congenital aortic stenosis in adults: preliminary results of a multicentre study
Authors: Keuning Z , Meccanici F , Sneep F M , Hirsch A , Takkenberg J J M , Van Den Bosch A E , Roos-Hesselink J W
Published: October 2022

Background

Congenital aortic stenosis (ConAoS) accounts for 4–8% of all congenital cardiac diagnosis and is associated with important morbidity and mortality. However, little is known about the natural history of ConAoS in adult patients. We aim to evaluate disease progression of ConAoS in young adults, describing both ventricular and valvular characteristics.

Methods

For this retrospective multicentre cohort study, data of one centre is collected up till now. We included ConAoS patients with a valvular stenosis (Vmax ≥2.5 m/s) aged 18–55 years from the Dutch CONCOR national registry from 2001 until 2019, excluding patients with prior aortic valve replacement (AVR) or severe aortic regurgitation. Echocardiographic data was used to determine presence of left ventricular (LV) hypertrophy (LV mass index >115 g/m2 in males and >95 g/m2 in females) and measure aortic jet velocity.

Results

A total of 92 patients (64% male, median age 24 years) were included with a median follow-up duration of 6.3 years (IQR 2.9–13.3). The median aortic jet velocity was 3.3 m/s (IQR 2.7–3.9) at baseline and significantly increased over time to 3.6 m/s (IQR 2.8–4.3) (p<0.001). LV hypertrophy was present in 33% of the patients and 17% of the patients showed signs of concentric remodelling at baseline. AVR was performed in 32 patients (35%, mean age 36±11 years) and one patient died during follow-up. Figure 1 shows significant differences in intervention-free survival for different AS severity groups (p<0.001) and presence of LV hypertrophy (p=0.02).

Conclusion

Although the progression rate of ConAoS seems to be slow, it is often necessary to perform AVR at a relatively young age. After data collection is completed, this study will enable us to identify risk factors for rapid disease progression and further improve current treatment protocols.

Funding Acknowledgement

Type of funding sources: Foundation. Main funding source(s): Dutch Heart Foundation, Hartekind
Mortality in patients with Ebstein anomaly: a two-country, population-based cohort study including 530 patients
Authors: Eckerstrom F , Hjortdal V E , Dellborg M , Eriksson P , Mandalenakis Z
Published: October 2022

Background

Low birth prevalence and referral bias constitutes significant obstacles in extending our knowledge regarding the natural history of Ebstein anomaly (EA).

Purpose

In an extensive two-country register-based collaboration we aimed to investigate the survival in EA patients with respect to associated congenital cardiac malformations

Methods

Patients born in the period 1970–2017 and diagnosed with EA were identified in Danish and Swedish nationwide medical registries and subsequently merged. Each EA patient was matched by birth year and gender with ten controls without congenital heart disease from the general population. Cox proportional hazard regression model and Kaplan-Meier survival function were used to compute risk of mortality (hazard ratio) and survival. Follow-up started at birth and patients became risk at time of EA diagnosis.

Results

We included 530 patients and 5,300 matched controls; median follow-up was 10.5 years (interquartile range: 3.5–20.9). In the total EA cohort, 43% (n=228) underwent cardiac surgery, predominantly EA-related surgery (81%). Approximately half of the cohort had an isolated EA (n=248), 11% (n=57) had a concomitant atrial septal defect only, and 42% (n=225) other associated congenital cardiac malformations. The 35-year survival in patients with isolated EA was 93%, superior compared with patients with concomitant atrial septal defect (ASD) (83%) and patients with other associated congenital cardiac malformations (72%), Figure 1. Patients with isolated non-operated EA had a risk of mortality of 25.6 (95% confidence interval (CI): 9.0–72.6) compared with the general population. Correspondingly, the risk in patients with surgically managed isolated EA was 21.7 (95% CI: 3.9–118.5). Patients with non-operated EA and a concomitant ASD (only) revealed a 10-fold increased risk of mortality (95% CI: 1.5–75.1) compared with the general population. The risk of mortality was substantially higher in both non-operated and operated EA patients with other associated congenital cardiac malformations, HR of 106.3 (95% CI: 31.8–335.4) and 42.6 (95% CI: 18.5–97.8), respectively.

Conclusions

Concomitant congenital cardiac malformations are common in EA patients and have major influence on survival, irrespective of treatment pathway (surgical versus non-surgical). Nonetheless, isolated non-operated EA patients have a 25-fold increased risk of mortality compared with the general population. Interestingly, concomitant ASD in non-operated patients seems protective in terms of risk of mortality.

Funding Acknowledgement

Type of funding sources: Foundation. Main funding source(s): Novo Nordisk Foundation.
Myocardial damage in patients with immunological diseases evaluated by cardiovascular magnetic resonance imaging
Authors: Sanchez Martinez M , Hurtado Duarte A M , Chango Azanza D , Chapa Ibarguengoitia M , Rosales Uvera S G
Published: October 2022

Background

Autoimmune diseases represent a family of pathologies with common pathogenic mechanisms and occur as consequence of loss of physiological tolerance to autoantigens. Cardiovascular involvement continues to be the main cause of death in these patients, the cardiologist should be able to make an early diagnosis of heart damage. Cardiac magnetic resonance imaging is a non-invasive method that can be used to assess morphological and functional integrity, to accurately determine if there is involvement in these patients and thus perform the necessary medications to improve their prognosis.

Purpose

To determine the presence of myocardial damage by cardiac magnetic resonance imaging in patients with immunological diseases.

Methods

Non-experimental quantitative longitudinal retrospective descriptive study of magnetic resonance imaging studies performed in patients with autoimmune diseases. The images were made in a 1.5T General Electric resonator and analysed in the Cardiac VX Cardiac Analysis and Reporting interpretation system by cardiologists and imaging specialists in cardiovascular imaging.

Results

We reviewed 48 cardiac magnetic resonance imaging studies (mean age 43 years SD ±16 years, 42 women and 6 men), the most prevalent autoimmune diseases were: lupus erythematosus systemic (31.3%), mixed connective tissue disease (20.8%), rheumatoid arthritis (12.5%), antiphospholipid antibody syndrome (10.4%), systemic sclerosis (10.4%). The alterations found were late gadolinium enhancement (77.08%), valve disease (64.5%), decreased left ventricular ejection fraction (56.25%) and decreased right ventricular fraction (69%) and pericardium involvement (56%).

20.8% had a positive myocarditis protocol, of which 8.3% were chronic events/sequelae of myocarditis and 12.5% were acute events. T2W ratio ≥2 presented in 27.1%, without meeting the criteria for acute myocarditis and ruling out an acute ischemic event.

Conclusion

Due to its significant ability to detect myocardial damage, before cardiac dysfunction becomes clinically manifest, CMR is an excellent diagnostic tool for risk stratification and individualization of treatment in patients with autoimmune diseases.

Funding Acknowledgement

Type of funding sources: None.
Is impaired exercise capacity associated with higher risk of mortality in adults with congenital heart disease?
Authors: Wikner A , Sandstrom A , Rinnstrom D , Christersson C , Dellborg M , Nielsen N E , Sorensson P , Thilen U , Johansson B , Sandberg C
Published: October 2022

Background

Impaired exercise capacity is a common finding among adults with congenital heart disease (CHD) and it has previously been reported that impaired exercise capacity is associated with higher risk of mortality, hospitalisation and/or transplantation. However, previous reports have been single centre with small sample sizes and composite endpoints.

Purpose

The aim of present report was to analyse the association between mortality, as primary endpoint, and aerobic exercise capacity in adults with CHD.

Methods

Data on exercise capacity from all registered test assessed with bicycle ergometer were retrieved from the Swedish Registry of Congenital Heart Disease. Percent of predicted peak workload (%Wpred) was calculated according to sex, age and height and compared to a national reference material (n=1790, 58% men).

Results

2748 adults with CHD were included (41% women). Median age was 31.7 years (range 18–80.6 years) and mean %Wpred was 77±21.0%. At a median follow-up time of 5.3 years (range 0.02–36.5 years) 135 patients were deceased (4.9%). Moderately impaired exercise capacity (50–70% Wpred) (HR 3.0, p<0.001), severely impaired exercise capacity (<50% Wpred) (HR 9.7, p<0.001), NYHA class II (HR 4.4, p<0.001) and NYHA class III–IV (HR 12.6, p<0.001) was associated with increased risk of mortality in univariable cox regression. In multi-variable Cox regression exercise capacity <50% Wpred (HR 3.0, 95% CI [1.5–5.8], p<0.001), NYHA class II (HR 2.8, 95% CI [1.6–5-1], <0.001) and NYHA class III–IV (HR 8.0, 95% CI [4.2–15.1] p<0.001) was associated with higher mortality risk adjusted for sex and diagnosis.

Conclusions

Our findings show that exercise capacity <50% and NYHA class ≥II are both associated with increased risk of mortality, regardless of diagnosis.

Funding Acknowledgement

Type of funding sources: Other. Main funding source(s): The Swedish Heart-Lung Foundation
Heart failure and long-term prognosis in patients with congenital heart disease: a nationwide, register-based cohort study
Authors: Bergh N , Skoglund K , Fedchenko M , Bollano E , Eriksson P , Dellborg M F , Giang Kok W , Mandalenakis Z
Published: October 2022

Introduction

Heart failure (HF) is associated with high morbidity and severe prognosis. The aim of this study was to investigate the risk of HF in patients with congenital heart disease (CHD) and to determine the prognosis after HF diagnoses in patients with CHD compared to HF controls without CHD.

Methods

We identified 89,532 patients with CHD born between 1930 and 2017 from the National Patient Register in Sweden and matched with 10 controls without CHD by gender and birth year from the National Population Register. The study population was followed-up until 2017, through the Swedish Patient Register and Cause of Death Register.

Results

Altogether, 7,013 (7.8%) patients with CHD and 9,681 (1.1%) controls developed HF. The overall incidence rate of HF was 31.6 per 104 person-years in patients with CHD and 4.0 per 104 person-years in controls. The mean age for HF diagnoses was 40.3 (± 28.8) in patients with CHD and 66.4 (± 13.6) for controls. The risk for developing HF was significantly higher in complex than non-complex CHD lesion groups, HR 28.7 (26.5–31.0) and HR 6.9 (6.7–7.2) respectively. Mortality incidence rate was twice as high in controls with HF compared to CHD with HF (IR 9.7 (9.4–10.0) and 4.74 (4.6–4.9), respectively). The mortality incidence was higher within all age groups except for 18–39 years and there was no difference between complex and non complex lesions

Conclusion

One out of thirteen CHD patients will develop HF. The highest risk for HF is within CHD patients with complex lesions. CHD patients develops HF at a considerably younger age, reflecting their slightly better prognosis after development of HF compared to controls with HF.

Funding Acknowledgement

Type of funding sources: None.
Lymphopenia and immunoglobulin deficiency in adult patients with a Fontan circulation
Authors: Possner M , Bonassin Tempesta F , Santos Lopes B , Meier L , Babic D , Attenhofer Jost C , Greutmann M
Published: October 2022

Background

Patients with a functionally univentricular heart and a Fontan palliation are prone to a wide range of extra-cardiac complications. Lymphopenia and immunoglobulin deficiency are insufficiently characterized in this population.

Purpose

The aim of this study was to analyze prevalence and associations of lymphopenia and immunoglobulin deficiency in a cohort of adult Fontan patients.

Methods

Ninety-five consecutive patients with a Fontan circulation that were seen at our institution between 2011 and 2021 were screened. Laboratory results and clinical characteristics were extracted from the patient's charts.

Results

Fifty-five patients (47% male) underwent evaluation of lymphocyte and/or immunoglobulin subpopulations at a mean age of 28.9±9.7 years. Baseline characteristics are depicted in the Table. Seven patients (7/55, 12.7%) had immunoglobulin G (IgG) levels below the lower limit of normal (i.e. <7.0 g/l). Patients with IgG deficiency had a higher prevalence of protein-losing enteropathy (43% versus 4%, p=0.001). Fifteen patients (15/52, 28.8%) had lymphocyte counts below the lower limit of normal (i.e. <850/μl). Lymphocyte counts and lymphocyte subpopulations were comparable in groups with and without protein-losing enteropathy. There was a moderately positive correlation between absolute lymphocyte counts and IgG levels (r=0.301, p=0.032). In a subgroup of 24 patients that underwent liver biopsy, there was no difference in terms of absolute lymphocyte counts and immunoglobulin subpopulations in patients with a low (i.e. 1–2) and high (i.e. 3–4) congestive hepatic fibrosis score.

Conclusion

IgG deficiency is present in 13% of patients with a Fontan circulation and seems to be associated with the presence of protein-losing enteropathy. Lymphopenia is more common and occurs in a quarter of all patients with a Fontan circulation; however, its etiology is likely more complex and multifactorial.

Funding Acknowledgement

Type of funding sources: None.
Adult outcomes of complex pulmonary atresia, ventricular septal defect and major aortopulmonary collaterals following repair during childhood, a single centre experience
Authors: Chambault A L , Drury N E , Poole E , Jones T , Khan N , Hudsmith L , Bowater S , Arif S , Botha P , Clift P
Published: October 2022

Background

Complex pulmonary atresia with a ventricular septal defect and major aortopulmonary collateral arteries (PA VSD MAPCA) is a rare and complex form of congenital heart disease. Staged surgical palliation is carried out in childhood, and in expert centres, is associated with good medium term survival. Adult outcomes following transition to adult care are not known.

Purpose

To establish adult outcomes of PA VSD MAPCA following repair during childhood in our institution

Methods

Review of clinical records for outcome data including life status, NYHA class, employment status, re-intervention in adult life, arrhythmia, pregnancy, exercise capacity and mode of death, where known. We predefined our population into two groups based on their status at transition to adult care, A) complete repair with RV-PA conduit and VSD closed and B) repaired but VSD left open.

Results

188 patients from our series have transitioned to adult services. As many were tertiary referrals (nationally and internationally) the local follow up population was 53 adults (54.7% male), mean age of those alive under follow up is 27.2±6.5 years, with a mean follow up of 25.3±6.1 years. There were 43 in group A and 10 in group B. Mortality in the total population was 18.9% (10/53), group A (16.3%), group B (30.0%), mean age at death 37.5±10.3 years and 29.9±8.4 years, 6/10 deaths were sudden. 22.6% of the whole group had DiGeorge/22q11 microdeletion, with a learning deficit in 32.7% of the cohort, where this could be assessed. Reinterventions included RV-PA conduit change, coil occlusion, pacemaker implantation, ICD implantation and ablation procedures, overall 39.6% underwent reintervention in adult life. Pregnancy occurred in 33.3% of 24 female patients. 50% of individuals were found to have undertaken some form of paid work and of these 11.5% had DiGeorge.

Conclusions

Complex PA VSD MAPCA is associated with good late survival with late attrition including sudden death and heart failure. 22q11 is associated with learning deficit and consequent lack of employment. Non 22q11 have good educational outcomes and there is less observed deficit regarding employment. Successful pregnancy is possible within this population.

Funding Acknowledgement

Type of funding sources: None.
Adults with congenital heart disease and COVID-19
Authors: Langes N , Maurer S J , Meierhofer C , Nagdyman N , Bourier F , Holdenrieder S , Ewert P , Tutarel O
Published: October 2022

Background

Adults with congenital heart disease (ACHD) are considered at increased risk for complications due to COVID-19, especially in those with cyanosis or heart failure. However, data regarding the rate of infection as well as regarding a myocardial involvement of a COVID-19 infection in ACHD patients are currently lacking.

Purpose

To study the rate of COVID-19 infections in ACHD patients from a tertiary centre as well as the rate of myocardial involvement of a COVID-19 infection.

Methods

All consecutive ACHD patients (over the age of 18 years) who attended the outpatient clinic of a tertiary centre from January 2021 to June 2021 were eligible to participate. Demographic data, as well as medical/surgical data including a history of a COVID-19 infection were collected. All patients had an antibody test for COVID-19. Patients with a positive antibody test were offered cardiovascular magnetic resonance imaging (CMR).

Results

Altogether, 420 patients (44.8% female, mean age 36.4±11.6 years) were included in the study. Congenital heart defect (CHD) complexity according to the Bethesda classification was simple in 96 (22.9%), moderate in 186 (44.3%), complex in 117 (27.9%), and miscellaneous in 21 (5.0%) patients. A positive antibody test for COVID-19 was present in 28 (6.7%) patients (CHD complexity: simple n=5, moderate n=14, severe n=7, miscellaneous n=2). Out of these 28 patients, 14 had no symptoms at all. Those with symptoms had mainly mild symptoms and were all managed in the outpatient setting. While 11 patients (39.3%) were not at all aware of their infection, 17 already knew that they had COVID-19. There was no significant difference between ACHD patients with a positive test vs those with a negative test regarding age, gender, New York Heart Association class, or complexity of CHD. Out of the 28 patients with a positive antibody test, 14 agreed to a CMR. A myocardial involvement, i.e. signs of active or healed myocarditis, was not present in any of these.

Conclusions

In this single-centre study, 6.7% of ACHD patients attending the outpatient clinic had positive antibodies for COVID-19. Out of these, 50% were asymptomatic and 39.3% were not aware of their infection. A myocardial involvement was not found in any of the patients that underwent a CMR. These results indicate a large number of undetected cases of COVID-19 in the ACHD population and offer reassurance that in the vast majority of cases the infection has a mild course.

Funding Acknowledgement

Type of funding sources: Public Institution(s). Main funding source(s): Bayerisches Staatsministerium für Wissenschaft und Kunst
Effects of sacubitril/valsartan in patients with a systemic right ventricle
Authors: Fusco F , Scognamiglio G , Merola A , Iannuzzi A , Palma M , Borrelli N , Barracano R , Correra A , Ciriello G D , Grimaldi N , Colonna D , Romeo E , Sarubbi B
Published: October 2022

Background

Sacubitril/valsartan has been proved to reduce mortality in heart failure and reduced ejection fraction (EF) and is currently recommended as first-line therapy. However, effects in patients with a systemic right ventricle (sRV) have not been systematically investigated yet.

Purpose

We aimed to assess safety and efficacy of sacubitril/valsartan in patients with a sRV

Methods

From September 2020 to April 2021, all patients with congenitally corrected transposition of the great arteries (TGA) or TGA after Senning/Mustard repair attending our tertiary centre were prospectively enrolled. Inclusion criteria were: age ≥18 years, 3-months of optimal medical therapy including ACEi/ARB and sRV EF≤40%. Patients with univentricular physiology, systolic blood pressure (SBP) <90 mmHg, glomerular filtration rate (GFR) <30 ml/min or K >5.5 mEq/L were excluded. SBP and blood samples were obtained at 1-month of treatment. Other clinical and echocardiographic variables were reassessed at 6 and 12-month follow-up and the medication was progressively up-titrated to the highest tolerated dose.

Results

Fifty-one patients (38±11 years, 60% male, 34% ccTGA) were included. Up to March 2022, 48 (92%) patients were reviewed after 6 months of therapy and 35 (68%) completed the first year of follow-up. Baseline patients' characteristics are summarized in Table 1. At 1 month, treatment did not impact on the serum potassium values (4.5±0.3 vs 4.4±0.3 mEq/L, p=0.9) and GFR (112±33 vs 112±31 ml/min, p=0.3), while SBP dropped significantly (119±13 vs 108±18 mmHg, p=0.003). Two patients ceased the treatment due to symptomatic hypotension during the first month. One patient developed a nephrotic syndrome at 4 months of follow-up, which was likely unrelated to the treatment. No other major adverse events were reported. One patient was lost to follow-up after 3 months. Despite no significant change in the NYHA class (p=0.9), the 6-minute walking distance increased significantly at 6-month (Table 2). Nt pro BNP values were significantly decreased at 6-month, and returned to baseline at 12-month. Improved sRV systolic function was demonstrated at 6 and 12-month by significant increase in fractional area change, RV global longitudinal strain and sRV EF measured with 3D echocardiography.

Conclusions

Our mid-term results showed that sacubitril/valsartan is well tolerated in patients with a sRV and leads to signficant improvement of sRV systolic function, supporting its use in this complex population.

Funding Acknowledgement

Type of funding sources: None.
The patient with Down syndrome and pulmonary arterial hypertension associated with congenital heart disease: from childhood to adulthood
Authors: Playan Escribano J , Garrido-Lestache E , Luna R , Alvarez Fuente M , Lopez-Meseguer M , Guillen Rodriguez I , Perez Penate G M , Sabate Rotes A , Elias Hernandez T , Lopez Ramon M , Rueda Soriano J , Perin F , Blanco I , Del Cerro Marin M J , Escribano Subias P
Published: October 2022

Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD) that significantly affects its prognosis. Down syndrome is a common comorbidity among patients with PAH associated with CHD. Children born in recent years with CHD have benefited from advances in pediatric cardiac surgery that have modified their evolution compared to adults with the same pathologies. Our objective was to know and compare the characteristics of the adult and pediatric population with DS and PAH associated with CHD.

REHAP and REHIPED are Spanish, voluntary, multicenter registries that include patients with PAH associated with CHD. REHAP started in 2007 and includes patients over 18 years of age. Patients diagnosed with PAH after this time were prospectively included, until 2021. Patients who were followed in the participating centers and diagnosed after January 1998 were retrospectively included in the registry. REHIPED started in 2009 and collects patients between 2 months and 18 years of age. Patients diagnosed with PAH after this time were prospectively included, until 2021. Patients who were followed and diagnosed after January 1998 were retrospectively included. 131 adults and 47 children with DS and PAH associated with CHD were analyzed. Their baseline characteristics, hemodynamics, treatment, and 10-year survival were compared.

Adult patients with DS presented more frequently complete atrioventricular canal defect as underlying heart disease and were mostly in Eisenmenger's situation (84%). In pediatric patients the largest group was post-repair PAH (50%). Functional class was similar in both groups, although adults presented significantly greater hemodynamic severity. Regarding medical treatment, no differences were observed between both groups: there was a very low use of prostanoids both at baseline and at follow-up, and a majority use of oral monotherapy. There were no significant differences in survival.

Corrective surgery in patients with DS has become more frequent over the years: adults are mostly in Eisenmenger's situation, while post-repair PAH is the most numerous group in children. Although adults show greater hemodynamic severity, survival is similar in both groups. Medical treatment, which showed no differences between pediatric and adult age, is very conservative, with most patients on oral monotherapy and rare use of prostanoids.

Funding Acknowledgement

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

Background

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

Purpose

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

Methods

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

Results

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

Conclusion

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

Funding Acknowledgement

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

Introduction and aims

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

Material and methods

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

Results

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

A maximum of 14% of adolescents were interested in acquiring transitional skills. Larger proportions of adolescents perceived the relevant transitional skills as insufficient, from 21% to 83%.

Conclusion

The preferences for disease-related information of adolescents with CHD are mainly related to medical topics and tend to decrease with increasing age. This motivates timely gauging of individual information needs and delivering disease-related information, ideally in the early teens. Furthermore, preferences varied according to gender, with more information requested by girls.

Adolescents expressed minimal needs to acquire transitional skills despite the low self-perceived skills levels. This paradox requires further research to enable the adaptation of transition interventions.

Funding Acknowledgement

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

Introduction

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

Purpose

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

Methods

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

Results

Our cohort was characterised by high BNP mean values (382 pg/mL), poor exercise capacity (260 mt at 6MWT, peak VO2 11 mL/kg/min at CPET), right ventricular dysfunction and severe PH at right heart catheterization (cardiac index 2.3 L/min/mq, mean pulmonary artery pressure 48 mmHg, pulmonary vascular resistance 12 WU). Sixty-nine percent (70/103) of the cohort had significant TR. Thirty percent of patients (30/103) died during follow-up.

At Cox univariate regression, moderate to severe TR was significantly associated with higher risk of death (HR 4.48, p=0.004) (Figure 1). At bivariate analysis combining TR grade and TAPSE/sPAP, significant TR showed to be associated with higher risk of death (HR 3.59, p=0.05). In particular, the combination of normal TAPSE and mild TR identified a subgroup of patients who had no events during the follow-up period (Figure 2).

At echocardiography patients with moderate-to-severe TR had more peripheral venous congestion, worse right ventricular function (TAPSE and TAPSE/sPAP), lower stroke volume index. The presence of moderate-to-severe TR promoted negative remodelling of the right atrium and of the right ventricle, which was more spherical, hypertrophic and stiff. Exercise capacity of patient with significant TR was significantly reduced both at CPET and at 6MWT.

Conclusions

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

Funding Acknowledgement

Type of funding sources: None.