bluuebunny/crossref_metadata_2025_split huggingface dataset:
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

Abstract

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 &gt;115 g/m2 in males and &gt;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&lt;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&lt;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

Abstract

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

Abstract

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

Abstract

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&lt;0.001), severely impaired exercise capacity (&lt;50% Wpred) (HR 9.7, p&lt;0.001), NYHA class II (HR 4.4, p&lt;0.001) and NYHA class III–IV (HR 12.6, p&lt;0.001) was associated with increased risk of mortality in univariable cox regression. In multi-variable Cox regression exercise capacity &lt;50% Wpred (HR 3.0, 95% CI [1.5–5.8], p&lt;0.001), NYHA class II (HR 2.8, 95% CI [1.6–5-1], &lt;0.001) and NYHA class III–IV (HR 8.0, 95% CI [4.2–15.1] p&lt;0.001) was associated with higher mortality risk adjusted for sex and diagnosis.

Conclusions
Our findings show that exercise capacity &lt;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

Abstract

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

Abstract

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. &lt;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. &lt;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

Abstract

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

Abstract

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

Abstract

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) &lt;90 mmHg, glomerular filtration rate (GFR) &lt;30 ml/min or K &gt;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.