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Background: Heart failure (HF) results in persistent risk and long-term comorbidities. This is particularly true for patients with lifelong HF sequelae of cardiovascular disease such as patients with congenital heart disease (CHD)., Purpose: We developed hART (heart failure Attentive Risk Trajectory), a deep-learning model to predict HF trajectories in CHD patients., Methods: hART is designed to capture the contextual relationships between medical events within a patient's history. It is trained to predict future HF risk by using the masked self-attention mechanism that forces it to focus only on the most relevant segments of the past medical events., Results: To demonstrate the utility of hART, we used a large cohort containing healthcare administrative data from the Quebec CHD database (137,493 patients, 35-year follow-up). hART achieves an area under the precision-recall of 28% for HF risk prediction, which is 33% improvement over existing methods. Patients with severe CHD lesion showed a consistently elevated predicted HF risks throughout their lifespan, and patients with genetic syndromes exhibited elevated HF risks until the age of 50. The impact of the birth condition decreases on long-term HF risk. The timing of interventions such as arrhythmia surgery had varying impacts on the lifespan HF risk among the individuals. Arrhythmic surgery performed at a younger age had minimal long-term effects on HF risk, while surgeries during adulthood had a significant lasting impact., Conclusion: Together, we show that hART can detect meaningful lifelong HF risk in CHD patients by capturing both long and short-range dependencies in their past medical events., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Author(s). Published by Elsevier B.V. All rights reserved.)

Together, heart failure and arrhythmia represent the most important cardiovascular sources of morbidity and mortality among adults with congenital heart disease (ACHDs). Although traditionally conceptualized as operating within 2 distinct clinical silos, these scenarios frequently coexist within the same individual; consequently the mechanistic, therapeutic, and prognostic overlap between them demands increased recognition. In fact, given the near ubiquity of heart failure and arrhythmia among ACHDs, there is perhaps no other arena within cardiology where this critical intersection is more frequently observed. Optimal care for ACHDs therefore requires a heightened awareness of the relevant interactions as well as the pharmacologic and interventional resources that are increasingly available to the treating cardiologist. This review explores and highlights the overlap between these 2 fields to recommend a parallel, yet interactive, multidisciplinary approach to clinical management. Congenital heart disease categories are broken down into their archetypal subtypes to highlight subtleties of the pathophysiology, evaluation, and therapeutic approach., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Published by Elsevier Inc.)

Aims: Hypertensive disorders of pregnancy (HDP) occur in 10% of pregnancies in the general population, pre-eclampsia specifically in 3-5%. Hypertensive disorders of pregnancy may have a high prevalence in, and be poorly tolerated by, women with heart disease., Methods And results: The prevalence and outcomes of HDP (chronic hypertension, gestational hypertension or pre-eclampsia) were assessed in the ESC EORP ROPAC (n = 5739), a worldwide prospective registry of pregnancies in women with heart disease.The overall prevalence of HDP was 10.3%, made up of chronic hypertension (5.9%), gestational hypertension (1.3%), and pre-eclampsia (3%), with significant differences between the types of underlying heart disease (P < 0.05). Pre-eclampsia rates were highest in women with pulmonary arterial hypertension (PAH) (11.1%), cardiomyopathy (CMP) (7.1%), and ischaemic heart disease (IHD) (6.3%). Maternal mortality was 1.4 and 0.6% in women with vs. without HDP (P = 0.04), and even 3.5% in those with pre-eclampsia. All pre-eclampsia-related deaths were post-partum and 50% were due to heart failure. Heart failure occurred in 18.5 vs. 10.6% of women with vs. without HDP (P < 0.001) and in 29.1% of those with pre-eclampsia. Perinatal mortality was 3.1 vs. 1.7% in women with vs. without HDP (P = 0.019) and 4.7% in those with pre-eclampsia., Conclusion: Hypertensive disorders of pregnancy and pre-eclampsia rates were higher in women with CMP, IHD, and PAH than in the general population. Adverse outcomes were increased in women with HDP, and maternal mortality was strikingly high in women with pre-eclampsia. The combination of HDP and heart disease should prompt close surveillance in a multidisciplinary context and the diagnosis of pre-eclampsia requires hospital admission and continued monitoring during the post-partum period., Competing Interests: Conflict of interest: None declared., (© The Author(s) 2022. Published by Oxford University Press on behalf of European Society of Cardiology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: Renin-angiotensin aldosterone system inhibitors (RAASi) are commonly used among patients hospitalized with a severe acute respiratory syndrome coronavirus 2 infection coronavirus disease 2019 (COVID-19). We evaluated whether continuation versus discontinuation of RAASi were associated with short term clinical or biochemical outcomes., Methods: The RAAS-COVID-19 trial was a randomized, open label study in adult patients previously treated with RAASi who are hospitalized with COVID-19 (NCT04508985). Participants were randomized 1:1 to discontinue or continue RAASi. The primary outcome was a global rank score calculated from baseline to day 7 (or discharge) incorporating clinical events and biomarker changes. Global rank scores were compared between groups using the Wilcoxon test statistic and the negative binomial test (using incident rate ratio [IRR]) and the intention-to-treat principle., Results: Overall, 46 participants were enrolled; 21 participants were randomized to discontinue RAASi and 25 to continue. Patients' mean age was 71.5 years and 43.5% were female. Discontinuation of RAASi, versus continuation, resulted in a non-statistically different mean global rank score (discontinuation 6 [standard deviation [SD] 6.3] vs continuation 3.8 (SD 2.5); P = .60). The negative binomial analysis identified that discontinuation increased the risk of adverse outcomes (IRR 1.67 [95% CI 1.06-2.62]; P = .027); RAASi discontinuation increased brain natriuretic peptide levels (% change from baseline: +16.7% vs -27.5%; P = .024) and the incidence of acute heart failure (33% vs 4.2%, P = .016)., Conclusion: RAASi continuation in participants hospitalized with COVID-19 appears safe; discontinuation increased brain natriuretic peptide levels and may increase risk of acute heart failure; where possible, RAASi should be continued., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Motivation: Recurrent neural networks (RNN) are powerful frameworks to model medical time series records. Recent studies showed improved accuracy of predicting future medical events (e.g., readmission, mortality) by leveraging large amount of high-dimensional data. However, very few studies have explored the ability of RNN in predicting long-term trajectories of recurrent events, which is more informative than predicting one single event in directing medical intervention., Methods: In this study, we focus on heart failure (HF) which is the leading cause of death among cardiovascular diseases. We present a novel RNN framework named Deep Heart-failure Trajectory Model (DHTM) for modelling the long-term trajectories of recurrent HF. DHTM auto-regressively predicts the future HF onsets of each patient and uses the predicted HF as input to predict the HF event at the next time point. Furthermore, we propose an augmented DHTM named DHTM+C (where "C" stands for co-morbidities), which jointly predicts both the HF and a set of acute co-morbidities diagnoses. To efficiently train the DHTM+C model, we devised a novel RNN architecture to model disease progression implicated in the co-morbidities., Results: Our deep learning models confers higher prediction accuracy for both the next-step HF prediction and the HF trajectory prediction compared to the baseline non-neural network models and the baseline RNN model. Compared to DHTM, DHTM+C is able to output higher probability of HF for high-risk patients, even in cases where it is only given less than 2 years of data to predict over 5 years of trajectory. We illustrated multiple non-trivial real patient examples of complex HF trajectories, indicating a promising path for creating highly accurate and scalable longitudinal deep learning models for modeling the chronic disease., Competing Interests: The authors have declared that no competing interests exist.

Background: Heart failure (HF) is the leading cause of death in adult patients with congenital heart disease (ACHD). No risk prediction model exists for HF hospitalization (HFH) for ACHD patients. We aimed to develop a clinically relevant one-year risk prediction system to identify ACHD patients at high risk for HFH., Methods: Data source was the Quebec CHD Database. A retrospective cohort including all ACHD patients aged 18-64 (1995-2010) was constructed for assessing the cumulative risk of HFH adjusting for competing risk of death. To identify one-year predictors of incident HFH, multivariable logistic regressions were employed to a nested case-control sample of all ACHD patients aged 18-64 in 2009. The final model was used to create a risk score system based on adjusted odds ratios., Results: The cohort included 29,991 ACHD patients followed for 648,457 person-years. The cumulative HFH risk by age 65 was 12.58%. The case-control sample comprised 26,420 subjects, of whom 189 had HFHs. Significant one-year predictors were age ≥ 50, male sex, CHD lesion severity, recent 12-month HFH history, pulmonary arterial hypertension, chronic kidney disease, coronary artery disease, systemic arterial hypertension, and diabetes mellitus. The created risk score ranged from 0 to 19. The corresponding HFH risk rose rapidly beyond a score of 8. The risk scoring system demonstrated excellent prediction performance., Conclusions: One eighth of ACHD population experienced HFH before age 65. Age, sex, CHD lesion severity, recent 12-month HFH history, and comorbidities constructed a risk prediction model that successfully identified patients at high risk for HFH., Competing Interests: Declaration of Competing Interest The authors report no relationships that could be construed as a conflict of interest., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: Hospital readmissions are common in adults with congenital heart disease (CHD) with heart failure (HF). We sought to examine the temporal risk of readmissions, the prompting diagnoses and their determinants within one-year after a first HF hospitalization in older CHD patients., Method: We assembled a cohort from the Quebec CHD database of patients whose first-ever HF hospitalization occurred after age 40. Subjects were followed from the first HF discharge to the earliest of all-cause readmission, death, or one-year post-discharge. The Fine and Gray model was used to measure the weekly readmission risks. The one-year period was categorized into different stages based on readmission risks. A multinomial logistic regression model was adopted to identify the determinants of patients falling in different readmission stages., Results: The one-year death-adjusted cumulative readmission risk was 48.8%. The absolute weekly risk of readmission was the highest at week 2, declined by 50% at week 8, and reached a plateau at week 27. Three phases-vigilance (1-8 weeks), transition (9-27 weeks), and plateau (28-52 weeks)-were identified, corresponding to high-risk, decreasing-risk, and stable-risk periods. Cardiovascular diseases accounted for 61.7%, 50.3%, and 43.2% of all readmissions during the three phases; systemic diseases accounted for 15.2%, 19.9%, and 31.2%. A history of past-12-month interventional procedures saw significantly decreased readmission risks. Longer hospital stays were associated with lower readmission risks at the vigilance phase only., Conclusion: Policy makers and caregivers should account for the change in risk over time, and phase-tailored strategies should be planned to effectively reduce readmissions., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: Nearly 90% of patients with adult congenital heart disease (ACHD) die after the age of 40 years, and heart failure (HF) is the most common cause of death. We aimed to characterize the association between an incident HF hospitalization (HFH) and mortality and to identify the predictors of 1-year postdischarge mortality after incident and repeated HFHs, respectively., Methods: Patients with ACHD aged ≥40 years between 2000 and 2010 were identified from the Québec CHD database. We conducted a propensity score-matched study to explore the association between an incident HFH and mortality. We performed Bayesian model averaging to identify the predictors of 1-year postdischarge mortality with a posterior probability ≥50% considered to be evidence of a significant association., Results: The mortality hazard ratio was high at 6.01 (95% CI, 4.02-10.72) within 1-year postdischarge, decreasing significantly but entering an elevated equilibrium until year 4 with a continued 3-fold increase in death. Kidney dysfunction (hazard ratio, 2.28 [95% credible interval, 1.59-3.28], posterior probability, 100.0%) and a history of ≥2 HFHs in the past 12 months (hazard ratio, 1.77 [95% credible interval, 1.18-2.66], posterior probability: 82.2%) were the most robust predictors of 1-year mortality after incident and repeated HFHs, respectively., Conclusions: In patients with ACHD aged ≥40 years, incident HFH was associated with high mortality risk at 1 year, declining but remaining elevated for 4 years. Kidney dysfunction was a potent predictor of 1-year mortality risk after incident HFHs. Repeated HFHs further increased mortality risk. These observations should inform early risk-tailored health services interventions for monitoring and prevention of HF and its associated complications in older patients with ACHD.

To summarise existing heart failure (HF) risk prediction models and describe the risk factors for HF-related adverse outcomes in adult patients with congenital heart disease (CHD). We performed a systematic search of MEDLINE, EMBASE and Cochrane databases from January 1996 to December 2018. Studies were eligible if they developed multivariable models for risk prediction of decompensated HF in adult patients with CHD (ACHD), death in patients with ACHD-HF or both, or if they reported corresponding predictors. A standardised form was used to extract information from selected studies. Twenty-five studies met the inclusion criteria and all studies were at moderate to high risk of bias. One study derived a model to predict the risk of a composite outcome (HF, death or arrhythmia) with a c-statistic of 0.85. Two studies applied an existing general HF model to patients with ACHD but did not report model performance. Twenty studies presented predictors of decompensated HF, and four examined patient characteristics associated with mortality (two reported predictors of both). A wide variation in population characteristics, outcome of interest and candidate risk factors was observed between studies. Although there were substantial inconsistencies regarding which patient characteristics were predictive of HF-related adverse outcomes, brain natriuretic peptide, New York Heart Association class and CHD lesion characteristics were shown to be important predictors. To date, evidence in the published literature is insufficient to accurately profile patients with ACHD. High-quality studies are required to develop a unique ACHD-HF prediction model and confirm the predictive roles of potential risk factors., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Emerging heart failure (HF) concepts in the growing population of adults with congenital heart disease (ACHD) are reviewed in the following article with a focus on individuals with tetralogy of Fallot (TOF), the largest group of adults with repaired cyanotic congenital heart disease (CHD). In the first section, the changing epidemiology of CHD and HF in ACHD patients is described. We demonstrate the challenges health care providers face when caring for this unique population. Emphasis is placed on the importance and difficulty of identifying patients at risk for HF, of which TOF patients comprise a substantial subset, underscoring the benefits of specialized cardiac care. In the second portion of the article, we review underlying mechanisms of HF in adults with TOF. We elaborate on the wide-ranging etiologies of HF that reflect a confluence of factors related to native anatomic substrate, history of surgical intervention(s), and superimposed hemodynamic and/or ischemic burden to the right and left heart. We describe state-of-the-art imaging concepts as they apply to qualifying and quantifying acquired myocardial and valvular dysfunction in adults with repaired TOF. In the final part of the article, we review the current literature pertaining to the management of adults with repaired TOF. Specifically, we explore medical and surgical issues related to pulmonary valve replacement, arrhythmia management, and transplantation. Finally, we highlight current knowledge gaps and propose future directions of much-needed research that will improve the quality of care for this growing population., (Copyright © 2015 Elsevier Inc. All rights reserved.)

The impact of lifelong exposure to myocardial dysfunction in populations with congenital heart disease (CHD) is becoming increasingly recognized. Most children born with CHD now reach adulthood and the long-term sequelae of treatment are contributing to substantial comorbidity. The combination of structural changes present at birth with changes resulting from cardiac surgery can result in heart failure. This article reports on the current state of knowledge on the epidemiology of heart failure in this patient population., (Copyright © 2014 Elsevier Inc. All rights reserved.)

To summarise existing heart failure (HF) risk prediction models and describe the risk factors for HF-related adverse outcomes in adult patients with congenital heart disease (CHD). We performed a systematic search of MEDLINE, EMBASE and Cochrane databases from January 1996 to December 2018. Studies were eligible if they developed multivariable models for risk prediction of decompensated HF in adult patients with CHD (ACHD), death in patients with ACHD-HF or both, or if they reported corresponding predictors. A standardised form was used to extract information from selected studies. Twenty-five studies met the inclusion criteria and all studies were at moderate to high risk of bias. One study derived a model to predict the risk of a composite outcome (HF, death or arrhythmia) with a c-statistic of 0.85. Two studies applied an existing general HF model to patients with ACHD but did not report model performance. Twenty studies presented predictors of decompensated HF, and four examined patient characteristics associated with mortality (two reported predictors of both). A wide variation in population characteristics, outcome of interest and candidate risk factors was observed between studies. Although there were substantial inconsistencies regarding which patient characteristics were predictive of HF-related adverse outcomes, brain natriuretic peptide, New York Heart Association class and CHD lesion characteristics were shown to be important predictors. To date, evidence in the published literature is insufficient to accurately profile patients with ACHD. High-quality studies are required to develop a unique ACHD-HF prediction model and confirm the predictive roles of potential risk factors. [ABSTRACT FROM AUTHOR]

Patients with systemic morphological right ventricles (RVs), including congenitally corrected transposition of the great arteries and dextro-transposition of the great arteries with a Mustard or Senning atrial baffle repair, have a high likelihood of developing systemic ventricular dysfunction. Unfortunately, there are a limited number of clinical studies on the efficacy of medical therapy for systemic RV dysfunction. We performed a systematic review and meta-analysis to assess the effect of angiotensin-converting enzyme (ACE) inhibitors, angiotensin-receptor blockers (ARBs), beta blockers, and aldosterone antagonists in adults with systemic RVs. The inclusion criteria included age ≥18 years, systemic RVs, and at least 3 months of treatment with ACE inhibitor, ARB, beta blocker, or aldosterone antagonist. The outcomes included RV end-diastolic and end-systolic dimensions, RV ejection fraction, functional class, and exercise capacity. EMBASE, PubMed, and Cochrane databases were searched. The selected data were pooled and analyzed with the DerSimonian-Laird random-effects meta-analysis model. Between-study heterogeneity was assessed with Cochran's Q test. A Bayesian meta-analysis model was also used in the event that heterogeneity was low. Bias assessment was performed with the Newcastle-Ottawa Scale and Cochrane Risk of Bias Tool, and statistical risk of bias was assessed with Begg and Mazumdar's test and Egger's test. Six studies met the inclusion criteria, contributing a total of 187 patients; treatment with beta blocker was the intervention that could not be analyzed because of the small number of patients and diversity of outcomes reported. After at least 3 months of treatment with ACE inhibitors, ARBs, or aldosterone antagonists, there was no statistically significant change in mean ejection fraction, ventricular dimensions, or peak ventilatory equivalent of oxygen. The methodological quality of the majority of included studies was low, mainly because of a lack of a randomized and controlled design, small sample size, and incomplete follow-up. In conclusion, pooled results across the limited available studies did not provide conclusive evidence with regard to a beneficial effect of medical therapy in adults with systemic RV dysfunction. Randomized controlled trials or comparative-effectiveness studies that are sufficiently powered to demonstrate effect are needed to elucidate the efficacy of ACE inhibitors, ARBs, beta blockers, and aldosterone antagonists in patients with systemic RVs. [ABSTRACT FROM AUTHOR]

Background The attrition of right ventricle to pulmonary artery (RV-PA) grafts has been attributed in part to the body's immunologic response. We hypothesized that antibodies developed through blood transfusion, directed against the grafts, may result in accelerated degeneration and the need for re-intervention. Methods This is a population-based study of the province of Quebec. We included children born between January 1, 1987 to December 31, 2006 who were diagnosed with a cono-truncal anomaly and had an RV-PA graft. The patients were followed for transfusion exposure and RV-PA graft re-intervention. Time to re-intervention in those exposed versus non-exposed was analyzed using Cox regression. Analysis was done in two time periods, before and after the calendar year 2000, given the change in blood preparation in the province of Quebec. Results There were 413 patients who met the inclusion criteria of a cono-truncal disorder. Of the whole study population, 69% received a blood transfusion. Cox regression analysis showed that among patients who had the initial graft performed (n = 181) before year 2000, having 2 or more blood transfusion was associated with an nearly tripled risk of a re-intervention comparing to no blood transfusion (hazard ratio of 2.88; 95% confidence interval 1.05–7.91). In patients who had the initial graft performed after year 2000 (n = 232), the associated risk increase was 7-fold (hazard ratio of 7.01; 95% confidence interval 3.06–16.02). Kaplan-Meier analyses confirmed the significant difference in the re-intervention free survival probabilities between those who received 2 or more blood product transfusion and those who did not as well: prior to year 2000 (67.9% vs. 88.0% at 5 years, p = 0.0201) as well as after year 2000 (39.7% vs. 82.8% at 5 years, p < 0.0001). Conclusion In this population-based analysis, repeated blood product transfusion was associated with a significant increased risk of a need for RV-PA graft re-intervention. This data strongly suggest that repeated blood transfusion may adversely impact graft longevity. [ABSTRACT FROM AUTHOR]

Background Significant pulmonary regurgitation, declining right-sided ejection fraction, increased right ventricular (RV) volumes as well as left ventricular (LV) dysfunction have all been identified as predictors of poor outcomes in patients with congenital heart disease (CHD). The prognostic value of the cardiac output (CO) in these patients however has never been studied. Methods All consecutive ambulatory adult patients with CHD referred for magnetic resonance imaging (MRI) at the Montreal Children's Hospital between June 2007 and May 2009 were included. Right ventricular (RV) and left ventricular (LV) variables including end diastolic and end systolic volumes (EDV, ESV respectively), ejection fractions (EF) and regurgitant volumes were obtained. Cardiac index (CI) was calculated. Patients were followed for cardiac-related hospitalizations and cardiac interventions. Results Ninety-six patients were included. Median follow up was 3.9 ± 1.4 years. Nineteen percent of patients had a systemic CI < 2.4 l/min/m 2 . LVEDV, LVEF and RVEF were significantly diminished in the low CI group with a significant increase in RVESV and total regurgitant volume. Best predictors of low CI were LVEF (AUC = 0.74), RVEF (AUC = 0.71), total RV regurgitant volume (AUC = 0.64) and RVESV (AUC = 0.563). Low systemic CI was the best predictor of cardiac-related hospitalizations (hazard ratio 3.5, 95% confidence interval 1.5–8.5) and cardiac interventions (hazard ratio 2.2, 95% confidence interval 1.3–4.0), superior to LVEF, RVEF, total regurgitant volume and RVESV parameters. Conclusions In patients with congenital heart disease, cardiac index is the best predictor of cardiac hospitalizations and cardiac interventions. [ABSTRACT FROM AUTHOR]