Special Considerations for Mechanical Circulatory Support or Device Therapy in Adult Congenital Heart Disease Heart Failure.

Heart failure has become the leading cause of mortality in adult congenital heart disease (ACHD) patients after the fifth decade of life. There is scanty evidence supporting the use of guideline-directed medical therapy in ACHD, especially in systemic right ventricle or single ventricle physiology. In complex patients, diagnosing heart failure and timely referral for advanced therapies are challenging. Mechanical circulatory support has been significantly developed over the past decade and has recently emerged as a feasible therapeutic option for these patients. This review summarizes current evidence of mechanical circulatory support in this population, its potential uses, and challenges.

Adult Congenital Heart Disease Care in Canada: Has Quality of Care Improved in the Last Decade?

BACKGROUND: Patients with adult congenital heart disease (ACHD) are at increased risk of comorbidity and death compared with the age-matched population. Specialized care is shown to improve survival. The purpose of this study was to analyze current measures of quality of care in Canada compared with those published by our group in 2012. METHODS: A survey focusing on structure and process measures of care quality in 2020 was sent to 15 ACHD centres registered with the Canadian Adult Congenital Heart Network. For each domain of quality, comparisons were made with those published in 2012. RESULTS: In Canada, 36,708 patients with ACHD received specialized care between 2019 and 2020. Ninety-five cardiologists were affiliated with ACHD centres. The median number of patients per ACHD clinic was 2000 (interquartile range [IQR]: 1050, 2875). Compared with the 2012 results, this represents a 68% increase in patients with ACHD but only a 19% increase in ACHD cardiologists. Compared with 2012, all procedural volumes increased with cardiac surgeries, increasing by 12% and percutaneous intervention by 22%. Wait time for nonurgent consults and interventions all exceeded national recommendations by an average of 7 months and had increased compared with 2012 by an additional 2 months. Variability in resources were noted across provincial regions. CONCLUSIONS: Over the past 10 years, ACHD care gaps have persisted, and personnel and infrastructure have not kept pace with estimates of ACHD population growth. Strategies are needed to improve and reduce disparity in ACHD care relative to training, staffing, and access to improved care for Canadians with ACHD.

The Impact of the COVID-19 Pandemic Restrictions on the Provision of Adult Congenital Heart Disease Care Across Canada: A National Survey.

Background: The COVID-19 pandemic significantly impacted health care access across Canada with the reduction in in-person evaluations. The aim of the study was to examine the effects of the COVID-19 pandemic on access to health care services among the Canadian population with adult congenital heart disease (ACHD). Methods: All Canadian adult congenital heart affiliated centres were contacted and asked to collect data on outpatient clinic and procedural volumes for the 2019 and 2020 calendar years. A survey was sent detailing questions on clinic and procedural volumes and wait times before and after pandemic restrictions. Descriptive statistics were used with the Student t-test to compare groups. Results: In 2019, there were 19,326 ACHD clinic visits across Canada and only 296 (1.5%) virtual clinic visits. However, during the first year of the pandemic, there were 20,532 clinic visits and 11,412 (56%) virtual visits (P < 0.0001). There were no differences in procedural volumes (electrophysiology, cardiac surgery, and percutaneous intervention) between 2019 and 2020. The mean estimated wait times (months) before the pandemic vs the pandemic were as follows: nonurgent consult 5.4 ± 2.6 vs 6.6 ± 4.2 (P = 0.65), ACHD surgery 6.0 ± 3.5 vs 7.0 ± 4.6 (P = 0.47), electrophysiology procedures 6.3 ± 3.3 vs 5.7 ± 3.3 (P = 0.72), and percutaneous intervention 4.6 ± 3.9 vs 4.4 ± 2.3 (P = 0.74). Conclusions: During the pandemic and restrictions of social distancing, the use of virtual clinic visits helped to maintain continuity in ACHD clinical care, with 56% of ACHD visits being virtual. The procedural volumes and wait times for consultation and percutaneous and surgical interventions were not delayed.

Contexte: La pandémie de COVID-19 a eu des répercussions sur l'accès aux soins de santé partout au Canada, y compris une diminution des évaluations en personne. La présente étude visait à évaluer l'effet de la pandémie de COVID-19 sur l'accès aux soins de santé chez les adultes atteints de cardiopathie congénitale. Méthodologie: Nous avons communiqué avec tous les centres canadiens de prise en charge de la cardiopathie congénitale chez l'adulte et nous leur avons demandé de recueillir des données sur les consultations externes et le volume des interventions pour les années 2019 et 2020. Un sondage détaillé leur a été transmis sur les volumes de consultations et d'interventions et sur les temps d'attentes avant et après la mise en place de restrictions liées à la pandémie. Les groupes ont été comparés lors d'une analyse statistique descriptive utilisant le test t de Student. Résultats: En 2019, 19 326 consultations pour cause de cardiopathie congénitale chez l'vadulte ont été enregistrées au Canada, dont seulement 296 (1,5 %) ont eu lieu en mode virtuel. Au cours de la première année de la pandémie, 20 532 consultations ont été relevées; 11 412 (56 %) ont été menées virtuellement (p < 0,0001). Aucune différence n'a été observée dans le volume d'interventions (interventions en électrophysiologie, interventions chirurgicales et interventions percutanées) entre 2019 et 2020. Les temps d'attente moyens estimés en mois, avant et pendant la pandémie, étaient les suivants : consultations non urgentes, 5,4 ± 2,6 vs 6,6 ± 4,2 (p = 0,65); interventions chirurgicales, 6,0 ± 3,5 vs 7,0 ± 4,6 (p = 0,47); interventions en électrophysiologie, 6,3 ± 3,3 vs 5,7 ± 3,3 (p = 0,72); et interventions percutanées, 4,6 ± 3,9 vs 4,4 ± 2,3 (p = 0,74). Conclusion: Au cours de la pandémie et de la période où les restrictions de distanciation sociale étaient en vigueur, le recours aux consultations virtuelles dans les cliniques a contribué à la continuité des soins offerts aux adultes atteints de cardiopathie congénitale, puisque 56 % des visites se sont déroulées virtuellement. Le volume d'interventions n'a pas été touché et les temps d'attentes pour les consultations, les interventions percutanées et les interventions chirurgicales ne se sont pas allongés.

Impact of a quality improvement initiative with a dedicated anesthesia team on outcomes after surgery for adult congenital heart disease.

Objectives: A quality improvement initiative was introduced to the adult congenital cardiac surgery program at Toronto General Hospital in January 2016. A dedicated Adult Congenital Anesthesia and intensive care unit team was introduced within the cardiac group. The use of factor concentrates was introduced. The study compares perioperative mortality, adverse events, and transfusion burden before and after this process change. Methods: We performed a retrospective analysis of all adult congenital cardiac surgeries from January 2004 to July 2019. Two groups were analyzed: patients undergoing operation before and after 2016. The primary outcome was in-hospital mortality. One-year mortality and prevalence of key morbidities were analyzed as secondary outcomes. A separate analysis looked at patients who had and had not attended an anesthesia-led preassessment clinic. Results: In-hospital mortality was significantly reduced in patients undergoing operation after 2016 (1.1% vs 4.3%, P = .003) despite a higher risk profile. One-year mortality (1.3% vs 5.8%, P = .003) and ventilation times (5.5 hours [3.4-13.0] vs 6.3 hours [4.2-16.2], P = .001) were also reduced. The incidence of stroke and renal failure was similar between groups. Blood product exposure was comparable, but the incidence of chest reopening decreased (1.8% vs 4.8%, P = .022), despite more patients with multiple previous chest wall incisions, on anticoagulation, and with more complex cardiac anatomy. There were no significant outcome differences between those who did or did not attend the preassessment clinic. Conclusions: Both in-hospital and 1-year mortality were significantly reduced after the introduction of a quality improvement program, despite a higher risk profile. Blood product exposure remained unchanged, but there were less chest reopenings.

Factors associated with long-term outcomes in adult congenital heart disease patients with infective endocarditis: a 16-year tertiary single-centre experience.

OBJECTIVES: Studies concerning factors associated with long-term outcomes in adult congenital heart disease (ACHD) patients after infective endocarditis (IE) are scarce, while IE-related mortality in these patients remains a burden. We evaluated the factors associated with long-term survival in ACHD patients admitted for IE. METHODS: We performed a retrospective single-centre study of all ACHD patients admitted for IE to a tertiary cardiothoracic centre between 1999 and 2015. Underlying ACHD, detailed echocardiographic and clinical data, surgical treatment and long-term follow-up were analysed. RESULTS: We identified 151 ACHD patients admitted due to 176 episodes IE with 30-day, 6-month and 1-, 5- and 10-year survival of 95.4%, 92.7%, 92.7%, 84.7% and 75.6%, respectively. In a multivariable analysis, adjusted estimated probability of death was consistently higher after an IE episode among patients with complex as compared to simple/moderate ACHD: 10.6% vs 2.4% at 30 days, 15.0% vs 3.4% at 6 months and 1 year, 30.4% vs 7.8% at 5 years and 44.9% vs 13.1% at 10 years. Risk of death was higher among patients with prosthetic valve in comparison with those without (risk ratios 1.73-1.92). Surgical treatment was required in 76 (43.2%) episodes with 30-day mortality of 3.9%. Risk of death appeared to be lower than in the conservatively treated subgroup (risk ratios 0.71-0.78). CONCLUSIONS: We demonstrated satisfactory long-term survival in ACHD patients who were treated for IE in a tertiary cardiothoracic centre. Early mortality tended to be lower in the surgically treated subgroup. Factors negatively associated with long-term survival were complex ACHD and presence of prosthetic valve.

Cardiovascular Complications of Down Syndrome: Scoping Review and Expert Consensus.

Cardiovascular disease is a leading cause of morbidity and mortality in individuals with Down syndrome. Congenital heart disease is the most common cardiovascular condition in this group, present in up to 50% of people with Down syndrome and contributing to poor outcomes. Additional factors contributing to cardiovascular outcomes include pulmonary hypertension; coexistent pulmonary, endocrine, and metabolic diseases; and risk factors for atherosclerotic disease. Moreover, disparities in the cardiovascular care of people with Down syndrome compared with the general population, which vary across different geographies and health care systems, further contribute to cardiovascular mortality; this issue is often overlooked by the wider medical community. This review focuses on the diagnosis, prevalence, and management of cardiovascular disease encountered in people with Down syndrome and summarizes available evidence in 10 key areas relating to Down syndrome and cardiac disease, from prenatal diagnosis to disparities in care in areas of differing resource availability. All specialists and nonspecialist clinicians providing care for people with Down syndrome should be aware of best clinical practice in all aspects of care of this distinct population.

Risk Stratification for Sudden Cardiac Death in Repaired Tetralogy of Fallot.

There has been significant progress in the prevention of sudden cardiac death in repaired tetralogy of Fallot. Contemporary cohorts report greater survival attributable to improved surgical techniques, heart failure management, and proactive strategies for risk stratification and management of ventricular arrhythmias including defibrillator implantation and ablation technology. Over the last 25 years, our understanding of predictive risk factors has also improved from invasive and more limited measures to individualized risk prediction scores based on extensive demographic, imaging, electrophysiological, and functional data. Although each of these contemporary scoring systems improves prediction, there are important differences between the study cohorts, included risk factors, and imaging modalities that can significantly affect interpretation and implementation for the individual patient. In addition, accurate phenotyping of disease complexity and anatomic repair substantially modulates this risk and the mechanism of sudden death. Routine implementation of risk stratification within repaired tetralogy of Fallot management is important and directly informs primary prevention defibrillator implantation as well as consideration for proactive invasive strategies including ventricular tachycardia ablation and pulmonary valve replacement. Assessment and risk stratification by a multidisciplinary team of experts in adult congenital heart disease are crucial and critical. Although we have increased understanding, reconciliation of these complex factors for the individual patient remains challenging and often requires careful consideration and discussion with multidisciplinary teams, patients, and their families.

De grands progrès ont été réalisés pour prévenir la mort subite d'origine cardiaque chez les patients ayant une tétralogie de Fallot réparée (TFr). Dans les cohortes contemporaines, l'amélioration du taux de survie peut être attribuée à l'évolution des techniques chirurgicales, à la prise en charge de l'insuffisance cardiaque et à la mise en place de stratégies proactives pour la stratification du risque d'arythmies ventriculaires et pour leur prise en charge, notamment par l'implantation de défibrillateurs et l'ablation. Au cours de 25 dernières années, les moyens utilisés pour caractériser les facteurs de risque à valeur prédictive sont passés de mesures limitées et invasives à l'établissement de scores individualisés basés sur de grands corpus de données démographiques, électrophysiologiques, fonctionnelles et d'autres issues de l'imagerie. Bien que chacun de ces systèmes contemporains d'évaluation du risque permette de raffiner notre capacité prédictive, des différences importantes entre les cohortes à l'étude, les facteurs de risque considérés et les modalités d'imagerie peuvent influencer l'interprétation des scores et les soins prodigués à un patient en particulier. De plus, la description phénotypique exacte de la complexité de la maladie et de la réparation anatomique permet de moduler la stratification du risque de mort subite d'origine cardiaque et son mécanisme possible. Il importe que la stratification du risque fasse partie intégrante de la prise en charge de la TFr puisqu'elle oriente directement le choix de mettre ou non en place un défibrillateur en prévention primaire, et qu'elle fasse partie de l'équation lorsque des stratégies invasives proactives, comme l'ablation de la tachycardie ventriculaire ou le remplacement de la valve pulmonaire, sont envisagées. La mesure et la stratification du risque par une équipe multidisciplinaire d'experts en cardiopathies congénitales sont donc des étapes cruciales. Même si les connaissances se sont affinées au fil du temps, il peut être difficile de faire la synthèse de ces facteurs complexes dans le cas d'un patient en particulier. C'est pourquoi il faut bien souvent se tourner vers l'équipe multidisciplinaire, le patient et ses proches pour évaluer rigoureusement les options.

Tetralogy of Fallot Across the Lifespan: A Focus on the Right Ventricle.

Tetralogy of Fallot is a cyanotic congenital heart disease, for which various surgical techniques allow patients to survive to adulthood. Currently, the natural history of corrected tetralogy of Fallot is underlined by progressive right ventricular (RV) failure due to pulmonic regurgitation and other residual lesions. The underlying cellular mechanisms that lead to RV failure from chronic volume overload are characterized by microvascular and mitochondrial dysfunction through various regulatory molecules. On a clinical level, these cardiac alterations are commonly manifested as exercise intolerance. The degree of exercise intolerance can be objectified and aid in prognostication through cardiopulmonary exercise testing. The timing for reintervention on residual lesions contributing to RV volume overload remains controversial; however, interval assessment of cardiac function and volumes by echocardiography and magnetic resonance imaging may be helpful. In patients who develop clinically important RV failure, clinicians should aim to maintain a euvolemic state through the use of diuretics while paying particular attention to preload and kidney function. In patients who develop signs of cardiogenic shock from right heart failure, stabilization through the use of inotropes and pressor is indicated. In special circumstances, the use of mechanical support may be appropriate. However, cardiologists should pay particular attention to residual lesions that may impact the efficacy of the selected device.

De nombreuses techniques chirurgicales permettent aux patients présentant une tétralogie de Fallot (TF), une forme de cardiopathie congénitale, de survivre jusqu'à l'âge adulte. À l'heure actuelle, l'évolution naturelle de la TF corrigée est caractérisée par une insuffisance ventriculaire droite (VD) progressive attribuable à une régurgitation pulmonaire et à d'autres lésions résiduelles. Les mécanismes cellulaires sous-jacents qui mènent à l'insuffisance VD due à une surcharge volumique chronique sont caractérisés par une dysfonction microvasculaire et mitochondriale faisant intervenir diverses molécules régulatrices. Sur le plan clinique, ces atteintes cardiaques se manifestent par une intolérance à l'effort qui peut être évaluée au moyen d'une épreuve d'effort cardiorespiratoire, ce qui permet de faciliter l'établissement d'un pronostic. Le moment propice pour une réintervention en cas de lésions résiduelles contribuant à la surcharge volumique du ventricule droit demeure controversé; toutefois, il peut être utile d'évaluer régulièrement la fonction et les volumes cardiaques au moyen d'une échocardiographie et de tests d'imagerie par résonance magnétique. En présence d'une insuffisance VD cliniquement importante, les cliniciens doivent tenter de maintenir les patients dans un état euvolémique en utilisant des diurétiques, tout en accordant une attention particulière à la précharge et à la fonction rénale. Si les patients manifestent des signes de choc cardiogénique associé à une insuffisance cardiaque droite, il convient de leur administrer des inotropes et des vasopresseurs pour stabiliser leur état. Dans certains cas, l'utilisation d'un dispositif d'assistance mécanique peut être appropriée. Cependant, les cardiologues doivent être attentifs aux lésions résiduelles, car elles peuvent influencer l'efficacité de ce dispositif.

Updates in the management of congenital heart disease in adult patients.

INTRODUCTION: Adults with congenital heart disease represent a highly diverse, ever-growing population. Optimal approaches to management of problems such as arrhythmia, sudden cardiac death, heart failure, transplant, application of advanced therapies and unrepaired shunt lesions are incompletely established. Efforts to strengthen our understanding of these complex clinical challenges and inform evidence-based practices are ongoing. AREAS COVERED: This narrative review summarizes evidence underpinning current approaches to congenital heart disease management while highlighting areas requiring further investigation. A search of literature published in 'Medline,' 'EMBASE,' and 'PubMed' using search terms 'congenital heart disease,' 'arrhythmia,' 'sudden cardiac death,' 'heart failure,' 'heart transplant,' 'advanced heart failure therapy,' 'ventricular assist device (VAD),' 'mechanical circulatory support (MSC),' 'intracardiac shunt' and combinations thereof was undertaken. EXPERT OPINION: Application of novel technologies in the diagnosis and management of arrhythmia has and will continue to improve outcomes in this population. Sudden death remains a prevalent problem with many persistent unknowns. Heart failure is a leading cause of morbidity and mortality. Improved access to specialist care, advanced therapies and cardiac transplant is needed. The emerging field of cardio-obstetrics will continue to define state-of-the-art care for the reproductive health of women with heart disease.

An unusual pair: coronary artery fistula and coronary sinus ostium stenosis as a cause of refractory angina.

Background: Coronary fistula are rare and often present in early adulthood with symptoms of right heart overload from left to right shunting or ischaemia in the distal coronary bed due to coronary steal. Case summary: A 73-year-old lady with prior history of supraventricular tachycardia, dyslipidemia and a right coronary artery (RCA) to coronary sinus (CS) fistula, presented with progressive angina. She did not have evidence of ischaemia in the RCA territory on nuclear imaging, and cardiac computed tomography (CT) did not show coronary artery disease but revealed a significantly dilated CS and coronary venous tree. She was found to have CS ostial stenosis and, under transesophageal echocardiographic guidance, underwent successful balloon angioplasty of the CS ostium, with decompression of the coronary venous circulation and resolution of her angina. Discussion: Coronary fistula draining to the CS are rare, and association with CS ostial stenosis has been reported very infrequently. CS ostial stenosis can cause elevated coronary venous pressure, leading to decreased global coronary perfusion and symptoms of angina or heart failure. Previous case reports of coronary fistula and CS ostial stenosis were treated with either medical therapy or surgery, and our case is the first to our knowledge to report successful percutaneous treatment.

Heart Transplant Indications, Considerations, and Outcomes in Fontan Patients: Age-Related Nuances, Transplant Listing, and Disease-Specific Indications.

In the current era, 5%-10% of Fontan patients die or need a transplant in childhood, and approximately 50% will experience the same fate by age 40 years. Heart transplant (HTx) can be successful for selected children and adults with Fontan circulatory failure of any mechanism, with a 1-year post-transplant survival rate approaching 90% in children and 80% in the largest single-centre adult Fontan HTx experience. Protein-losing enteropathy and plastic bronchitis can be expected to resolve post-transplant, and limited data suggest patients with Fontan-associated liver disease who survive HTx can expect improvement in liver health. Early Fontan failure, within 12 months of Fontan completion, is not easily rescued by HTx, and late referrals and failure to refer adult patients remain problematic. Very little is known about the numbers of patients who are not referred, are turned down following assessment for HTx, or die on the waiting list-numbers that are needed to understand the complete picture of HTx in the Fontan population and to identify where best to focus quality-improvement efforts. Recent revisions to listing prioritization in Canada with considerations specific to the Fontan population aim to mitigate the fact that the status-listing criteria are not tailored to the congenital heart population. Transplanting high-risk children prior to Fontan completion, developing adult congenital heart disease transplant centres with expertise that can also offer combined heart-liver transplant when appropriate, and improving single-ventricle mechanical support options and criteria for both adults and children may help mitigate the early post-listing mortality.

Outcome and right ventricle remodelling after valve replacement for pulmonic stenosis.

BACKGROUND: Complications and need for reinterventions are frequent in patients with pulmonary valve stenosis (PVS). Pulmonary regurgitation is common, but no data are available on outcome after pulmonary valve replacement (PVR). METHODS: We performed a retrospective analysis of 215 patients with PVS who underwent surgical valvotomy or balloon valvuloplasty. Incidence and predictors of reinterventions and complications were identified. Right ventricle (RV) remodelling after PVR was also assessed. RESULTS: After a median follow-up of 38.6 (30.9-49.4) years, 93% of the patients were asymptomatic. Thirty-nine patients (18%) had at least one PVR. Associated right ventricular outflow tract (RVOT) intervention and the presence of an associated defect were independent predictors of reintervention (OR: 4.1 (95% CI 1.5 to 10.8) and OR: 3.6 (95% CI 1.9 to 6.9), respectively). Cardiovascular death occurred in 2 patients, and 29 patients (14%) had supraventricular arrhythmia. Older age at the time of first intervention and the presence of an associated defect were independent predictors of complications (OR: 1.0 (95% CI 1.0 to 1.1) and OR: 2.1 (95% CI 1.1 to 4.2), respectively). In 16 patients, cardiac magnetic resonance before and after PVR was available. The optimal cut-off values for RV volume normalisation were 193 mL/m2 for RV end-diastolic volume indexed(sensitivity 80%, specificity 64%) and 100 mL/m2 for RV end-systolic volume indexed(sensitivity 80%, specificity 56%). CONCLUSIONS: Previous RVOT intervention, presence of an associated defect and older age at the time of first repair were predictors of outcome. More data are needed to guide timing of PVR, and extrapolation of tetralogy of Fallot guidelines to this population is unlikely to be appropriate.

Fate of the liver in the survivors of adult heart transplant for a failing Fontan circulation.

The Fontan circulation has inherent long-term vulnerabilities such that adult Fontan patients now comprise the largest, most rapidly growing subgroup of adult congenital heart disease referred for transplant assessment. Almost all have Fontan Associated Liver Disease (FALD). There is an absence of mid to late hepatic outcome data after heart transplant alone. Therefore, we analyzed outcomes of survivors of heart only transplant in patients with failing Fontan circulation. Including all 10 of our adult Fontan patients surviving >1 year after isolated heart transplant, we report evolution of their clinical features, bloodwork, hemodynamic data, and liver ultrasound findings over a median of 4.7 years. Nonprogression of FALD, resolution of ascites and freedom from hepatocellular carcinoma in the mid-term highlight the outcomes in this selected group once normal cardiac output and venous pressures are established by heart transplant.

Outcomes and healthcare resource utilization in adult congenital heart disease patients with heart failure.

AIMS: While heart failure (HF) is a leading cause of death in adults with congenital heart disease (ACHD), few studies report contemporary outcomes after the first HF hospitalization. We examined outcomes of ACHD patients newly admitted for HF compared with ACHD patients without HF and the general HF population without ACHD. METHODS AND RESULTS: Using population databases from a single-payer health system from 1994 to 2018, ACHD patients newly admitted for HF were matched 1:1 to ACHD patients without HF (n = 4030 matched pairs). Similarly, ACHD patients newly admitted for HF were matched 1:1 to HF patients without ACHD (n = 4336 matched pairs). Patients with ACHD and HF (median age 68 years, 45% women) experienced higher mortality in short-term [30 day adjusted hazard ratio (HR) 4.68, 95% confidence interval (CI) 4.06, 5.43, P < 0.001], near-term (1 year HR 3.87, 95% CI 3.77, 4.92, P < 0.001), and long-term (24 year HR 1.59, 95% CI 1.13, 2.36, P = 0.008) follow-up. Patients with ACHD and HF had fewer baseline cardiovascular comorbidities than non-ACHD HF but demonstrated higher 30 day (HR 1.56, 95% CI 1.41, 1.73, P < 0.001), 1 year (HR 1.30, 95% CI 1.20, 1.40, P < 0.001), and 24 year (HR 2.40, 95% CI 1.73, 3.38, P < 0.001) mortality. Those with ACHD and HF also exhibited higher cardiovascular readmission rates at 30 days with HRs 9.15 (95% CI; 8.00, 10.48, P < 0.001) vs. ACHD without HF, and 1.71 (95% CI; 1.54, 1.85, P < 0.001) vs. HF without ACHD, and the higher readmission risk extended to 10 year follow-up. CONCLUSIONS: Adults with congenital heart disease patients with new HF have high risks of death and cardiovascular hospitalization, and preventative strategies to improve outcomes are urgently needed.

The Role of Ablation in Prevention of Recurrent Implantable Cardioverter Defibrillator Shocks in Patients With Tetralogy of Fallot.

BACKGROUND: Implantable cardioverter defibrillators (ICDs) are effective in preventing arrhythmic sudden cardiac death in patients with tetralogy of Fallot (TOF). Although ICD therapies for malignant ventricular arrhythmias can be life-saving, shocks could have deleterious consequences. Substrate-based ablation therapy has become the standard of care to prevent recurrent ICD shocks in patients with ischemic cardiomyopathy. However, the efficacy and safety of this invasive therapy in the prevention of recurrent ICD shocks in patients with TOF has not been well evaluated. METHODS: Records of a total of 47 consecutive TOF patients (mean age: 43.1 ± 13.2 years, male sex: n = 34 [72.3%]) who underwent ICD implantation for secondary prevention between 2000 and 2018 were reviewed. RESULTS: Twenty (42.6%) patients underwent invasive therapy (radiofrequency catheter ablation, n = 8; surgical ablation with pulmonary valve replacement, n = 12) before ICD implantation. Twenty-seven patients (57.4%) were managed noninvasively. During follow-up (median 80.5 [interquartile range, 28.5-131.0] months), 2 (10.0%) patients in the invasive group and 10 (37.0%) patients in the noninvasive group received appropriate ICD shocks (P = 0.036). Logistic regression analysis showed that invasive therapy was associated with a decreased risk of ICD shocks by 81.1% (odds ratio, 0.189; 95% confidence interval, 0.036-0.990; P = 0.049). Furthermore, invasive therapy was associated with decreased risk of the composite outcomes of ICD shock, death, cardiac transplantation, and hospital admission (odds ratio, 0.090; 95% confidence interval, 0.025-0.365; P = 0.013) compared with noninvasive therapy. CONCLUSIONS: Invasive substrate modification therapy was associated with a lower likelihood of ICD shocks and improvement of long-term outcomes in TOF patients.

CONTEXTE: Les défibrillateurs cardioverteurs implantables (DCI) sont efficaces pour prévenir la mort cardiaque subite provoquée par une arythmie chez les patients présentant une tétralogie de Fallot (TF). Bien que le traitement des arythmies ventriculaires malignes par DCI puisse sauver des vies, les chocs administrés peuvent avoir des conséquences délétères. L'ablation du substrat est devenue le traitement de référence pour prévenir l'administration à répétition de chocs par DCI chez les patients atteints d'une cardiomyopathie ischémique. L'efficacité et l'innocuité de ce traitement invasif pour prévenir l'administration de chocs répétés chez les patients présentant une TF n'ont toutefois pas été bien évaluées. MÉTHODOLOGIE: Nous avons examiné les cas consécutifs de 47 patients présentant une TF (âge moyen : 43,1 ± 13,2 ans; hommes : n = 34 [72,3 %]) ayant reçu un DCI en prévention secondaire entre 2000 et 2018. RÉSULTATS: Au total, 20 (42,6 %) patients ont subi un traitement invasif (ablation par cathéter par radiofréquence, n = 8; ablation chirurgicale et remplacement de la valve pulmonaire, n = 12) avant l'implantation d'un DCI. Vingt-sept patients (57,4 %) ont été pris en charge de façon non invasive. Au cours de la période de suivi (durée médiane de 80,5 [intervalle interquartile : 28,5 à 131,0] mois), 2 (10,0 %) patients du groupe ayant subi une intervention invasive et 10 (37,0 %) patients du groupe ayant subi une intervention non invasive ont reçu un choc approprié par DCI (p = 0,036). Les résultats de l'analyse par régression logistique montrent que le traitement invasif est associé à une réduction du risque de choc par DCI de 81,1 % (rapport des cotes : 0,189; intervalle de confiance à 95 % : de 0,036 à 0,990; p = 0,049). En outre, le traitement invasif est associé à une réduction du risque de survenue d'un des événements du paramètre d'évaluation composé, soit un choc administré par DCI, le décès, une transplantation cardiaque ou une hospitalisation (rapport des cotes : 0,090; intervalle de confiance à 95 % : de 0,025 à 0,365; p = 0,013) par rapport au traitement non invasif. CONCLUSIONS: La modification invasive du substrat a été associée à une probabilité plus faible de choc administré par DCI et à une amélioration des résultats à long terme chez les patients présentant une TF.

Role of amiodarone in the management of atrial arrhythmias in adult Fontan patients.

BACKGROUND: Patients with Fontan circulation are known to be at high risk for developing atrial tachyarrhythmias (AAs). Our objective was to examine the efficacy and safety of amiodarone in the management of ATs in adult Fontan patients. METHODS: Primary outcomes of this single-centre, retrospective study included freedom from AAs and incidence of adverse effects of amiodarone on Fontan patients. Heart failure (HF) events and composite outcomes of death from any cause, Fontan revision and heart transplantation were evaluated as secondary outcomes. Predictors of HF and discontinuing amiodarone were also evaluated. RESULTS: A total of 61 patients (mean age 31.6±11.3 years, 40.9% female), who were treated with amiodarone in between 1995 and 2018, were included. AAs free survival at 1, 3 and 5 years were 76.2%, 56.9% and 30.6%, respectively. During a median follow-up of 50.5 months, 34 (55.7%) patients developed side effects, and 20 (32.8%) patients discontinued amiodarone due to side effects. Thyroid dysfunction was the most common side effect (n=26, 76.5%), amiodarone-induced thyrotoxicosis (AIT) (n=16, 27.1%) being most common thyroid dysfunction. Young age (age <28.5 years) was associated with discontinuing amiodarone (HR 5.50, 95% CI 1.19 to 25.4, p=0.029). AIT significantly increased risk of HF (HR 4.82, 95% CI 1.71 to 13.6, p=0.003). CONCLUSIONS: Short-term efficacy of amiodarone in Fontan physiology is acceptable. However, long-term administration is associated with a reduction of efficacy and a significant prevalence of non-cardiac side effects. AIT is associated with exacerbation of HF. The judicious use of amiodarone administration should be considered in this population.

Augmentation of pulmonary blood flow and cardiac output by non-invasive external ventilation late after Fontan palliation.

OBJECTIVES: Although a life-preserving surgery for children with single ventricle physiology, the Fontan palliation is associated with striking morbidity and mortality with advancing age. Our primary objective was to evaluate the impact of non-invasive, external, thoraco-abdominal ventilation on pulmonary blood flow (PBF) and cardiac output (CO) as measured by cardiovascular magnetic resonance (CMR) imaging in adult Fontan subjects. METHODS: Adults with a dominant left ventricle post-Fontan palliation (lateral tunnel or extracardiac connections) and healthy controls matched by sex and age were studied. We evaluated vascular flows using phase-contrast CMR imaging during unassisted breathing, negative pressure ventilation (NPV) and biphasic ventilation (BPV). Measurements were made within target vessels (aorta, pulmonary arteries, vena cavae and Fontan circuit) at baseline and during each ventilation mode. RESULTS: Ten Fontan subjects (50% male, 24.5 years (IQR 20.8-34.0)) and 10 matched controls were studied. Changes in PBF and CO, respectively, were greater following BPV as compared with NPV. In subjects during NPV, PBF increased by 8% (Δ0.20 L/min/m2 (0.10-0.53), p=0.011) while CO did not change significantly (Δ0.17 L/min/m2 (-0.11-0.23), p=0.432); during BPV, PBF increased by 25% (Δ0.61 L/min/m2 (0.20-0.84), p=0.002) and CO increased by 16% (Δ0.47 L/min/m2 (0.21-0.71), p=0.010). Following BPV, change in PBF and CO were both significantly higher in subjects versus controls (0.61 L/min/m2 (0.2-0.84) vs -0.27 L/min/m2 (-0.55-0.13), p=0.001; and 0.47 L/min/m2 (0.21-0.71) vs 0.07 L/min/m2 (-0.47-0.33), p=0.034, respectively). CONCLUSION: External ventilation acutely augments PBF and CO in adult Fontan subjects. Confirmation of these findings in larger populations with longer duration of ventilation and extended follow-up will be required to determine sustainability of haemodynamic effects.

Safety and efficacy of non-vitamin K antagonist oral anticoagulants for prevention of thromboembolism in adults with systemic right ventricle: Results from the NOTE international registry.

BACKGROUND: Patients with systemic right ventricle (sRV), including transposition of great arteries (TGA) after atrial switch procedure and congenitally corrected transposition of great arteries (ccTGA), may require anticoagulation for thromboembolism (TE) prevention. In the absence of data on non-vitamin K antagonist oral anticoagulants (NOACs), vitamin K antagonists (VKAs) remain the agent of choice. We investigated the safety, efficacy and feasibility of NOACs treatment in adults with sRV in a worldwide study. METHODS: This is an international multicentre prospective study, using data from the NOTE registry on adults with sRV taking NOACs between 2014 and 2019. The primary endpoints were TE and major bleeding (MB). The secondary endpoint was minor bleeding. RESULTS: A total of 76 patients (42.5 ±â€¯10.0 years, 76% male) with sRV (74% TGA, 26% ccTGA) on NOACs were included in the study. During a median follow-up of 2.5 years (IQR1.5-3.9), TE events occurred in 3 patients (4%), while no MB episodes were reported. Minor bleeding occurred in 9 patients (12%). NOAC treatment cessation rate was 1.4% (95%CI:0.3-4%) during the first year of follow-up. All the patients with TE events had a CHA2DS2-VASc score ≥ 2 and impaired sRV systolic function at baseline. The total incidence of major events during follow-up was significantly lower compared to historical use of VKAs or aspirin before study inclusion (1.4% (95%CI:0.29-4%) vs 6,9% (95%CI:2.5-15.2%); p = .01). CONCLUSIONS: In this prospective study, NOACs appear to be well-tolerated, with excellent efficacy and safety at mid-term in patients with sRV.