The corona virus disease -2019 (COVID-19) is a recently described infectious disease caused by the severe acute respiratory syndrome corona virus 2 with significant cardiovascular implications. Given ...the increased risk for severe COVID-19 observed in adults with underlying cardiac involvement, there is concern that patients with pediatric and congenital heart disease (CHD) may likewise be at increased risk for severe infection. The cardiac manifestations of COVID-19 include myocarditis, arrhythmia and myocardial infarction. Importantly, the pandemic has stretched health care systems and many care team members are at risk for contracting and possibly transmitting the disease which may further impact the care of patients with cardiovascular disease. In this review, we describe the effects of COVID-19 in the pediatric and young adult population and review the cardiovascular involvement in COVID-19 focusing on implications for patients with congenital heart disease in particular.
The arrhythmia burden in tetralogy of Fallot, types of arrhythmias encountered, and risk profile may change as the population ages.
The Alliance for Adult Research in Congenital Cardiology (AARCC) ...conducted a multicenter cross-sectional study to quantify the arrhythmia burden in tetralogy of Fallot, to characterize age-related trends, and to identify associated factors. A total of 556 patients, 54.0% female, 36.8+/-12.0 years of age were recruited from 11 centers. Overall, 43.3% had a sustained arrhythmia or arrhythmia intervention. Prevalence of atrial tachyarrhythmias was 20.1%. Factors associated with intraatrial reentrant tachycardia in multivariable analyses were right atrial enlargement (odds ratio OR, 6.2; 95% confidence interval CI, 2.8 to 13.6), hypertension (OR, 2.3; 95% CI, 1.1 to 4.6), and number of cardiac surgeries (OR, 1.4; 95% CI, 1.2 to 1.6). Older age (OR, 1.09 per year; 95% CI, 1.05 to 1.12), lower left ventricular ejection fraction (OR, 0.93 per unit; 95% CI, 0.89 to 0.96), left atrial dilation (OR, 3.2; 95% CI, 1.5 to 6.8), and number of cardiac surgeries (OR, 1.5; 95% CI, 1.2 to 1.9) were jointly associated with atrial fibrillation. Ventricular arrhythmias were prevalent in 14.6% and jointly associated with number of cardiac surgeries (OR, 1.3; 95% CI, 1.1 to 1.6), QRS duration (OR, 1.02 per 1 ms; 95% CI, 1.01 to 1.03), and left ventricular diastolic dysfunction (OR, 3.3; 95% CI, 1.5 to 7.1). Prevalence of atrial fibrillation and ventricular arrhythmias markedly increased after 45 years of age.
The arrhythmia burden in adults with tetralogy of Fallot is considerable, with various subtypes characterized by different profiles. Atrial fibrillation and ventricular arrhythmias appear to be influenced more by left- than right-sided heart disease.
Despite the overall improvement in life expectancy of patients living with congenital heart disease (congenital HD), disparities in morbidity and mortality remain throughout the lifespan. ...Longstanding systemic inequities, disparities in the social determinants of health, and the inability to obtain quality lifelong care contribute to poorer outcomes. To work toward health equity in populations with congenital HD, we must recognize the existence and strategize the elimination of inequities in overall congenital HD morbidity and mortality, disparate health care access, and overall quality of health services in the context of varying social determinants of health, systemic inequities, and structural racism. This requires critically examining multilevel contributions that continue to facilitate health inequities in the natural history and consequences of congenital HD. In this scientific statement, we focus on population, systemic, institutional, and individual-level contributions to health inequities from prenatal to adult congenital HD care. We review opportunities and strategies for improvement in lifelong congenital HD care based on current public health and scientific evidence, surgical data, experiences from other patient populations, and recognition of implicit bias and microaggressions. Furthermore, we review directions and goals for both quantitative and qualitative research approaches to understanding and mitigating health inequities in congenital HD care. Finally, we assess ways to improve the diversity of the congenital HD workforce as well as ethical guidance on addressing social determinants of health in the context of clinical care and research.
Left ventricular (LV) systolic dysfunction has been observed in patients with repaired tetralogy of Fallot (TOF), although its clinical associations are unknown. Adults with repaired TOF were ...identified from 11 adult congenital heart disease centers. Clinical history was reviewed. Patients with pulmonary atresia were excluded. Echocardiograms were reanalyzed to estimate LV ejection fraction. LV function was defined as normal (LV ejection fraction ≥55%) or mildly (45% to 54%), moderately (35% to 44%), or severely (<35%) decreased. Right ventricular (RV) and LV dimensions and Doppler parameters were remeasured. Function of all valves was qualitatively scored. Of 511 patients studied, LV systolic dysfunction was present in 107 (20.9%, 95% confidence interval 17.4 to 24.5). Specifically, 74 (14.4%) had mildly decreased and 33 (6.3%) had moderately to severely decreased systolic function. Presence of moderate to severe LV dysfunction was associated with male gender, LV enlargement, duration of shunt before repair, history of arrhythmia, QRS duration, implanted cardioverter–defibrillator, and moderate to severe RV dysfunction. Severity or duration of pulmonary regurgitation was not different. In conclusion, LV systolic dysfunction was found in 21% of adult patients with TOF and was associated with shunt duration, RV dysfunction, and arrhythmia.
Cancer Risk in Congenital Heart Disease—What Is the Evidence? Cohen, Sarah; Gurvitz, Michelle Z.; Beauséjour-Ladouceur, Virginie ...
Canadian journal of cardiology,
December 2019, 2019-Dec, 2019-12-00, 20191201, Letnik:
35, Številka:
12
Journal Article
Recenzirano
As life expectancy in patients with congenital heart disease (CHD) has improved, the risk for developing noncardiac morbidities is increasing in adult patients with CHD (ACHD). Among these noncardiac ...complications, malignancies significantly contribute to the disease burden of ACHD patients. Epidemiologic studies of cancer risk in CHD patients are challenging because they require large numbers of patients, extended follow-up, detailed and validated clinical data, and appropriate reference populations. However, several observational studies suggest that cancer risks are significantly elevated in patients with CHD compared with the general population. CHD and cancer share genetic and environmental risk factors. An association with exposure to low-dose ionizing radiation secondary to medical therapeutic or diagnostic procedures has been reported. Patients with Down syndrome, as well as, to a lesser extent, deletion of 22q11.2 and renin-angiotensin system pathologies, may manifest both CHD and a predisposition to cancer. Such observations suggest that carcinogenesis and CHD may share a common basis in some cases. Finally, specific conditions, such as Fontan circulation and cyanotic CHD, may lead to multisystem consequences and subsequently to cancer. Nonetheless, there is currently no clear consensus regarding appropriate screening for cancer and surveillance modalities in CHD patients. Physicians caring for patients with CHD should be aware of this potential predisposition and meet screening recommendations for the general population fastidiously. An interdisciplinary and global approach is required to bridge the knowledge gap in this field.
Comme l’espérance de vie des patients atteints d’une cardiopathie congénitale augmente, le risque d’apparition de maladies non cardiaques est aussi en hausse chez les adultes qui sont atteints de ce type de cardiopathie. Parmi les complications non cardiaques, les affections malignes contribuent fortement au fardeau de la maladie dans cette population. La réalisation d’études épidémiologies sur le risque de cancer chez les patients atteints d’une cardiopathie congénitale présente des défis, car de telles études exigent un grand nombre de sujets qu’il faut suivre sur une longue période, des données cliniques détaillées et validées et des populations de référence appropriées. Malgré tout, plusieurs études observationnelles semblent indiquer que le risque de cancer est considérablement élevé chez les patients atteints d’une cardiopathie congénitale comparativement à la population générale. De fait, les facteurs de risque génétiques et environnementaux sont les mêmes pour la cardiopathie congénitale et pour le cancer. Une association avec l’exposition à de faibles doses de rayonnements ionisants secondaire aux démarches thérapeutiques ou diagnostiques a été rapportée. Les patients atteints du syndrome de Down ainsi que, dans une moindre mesure, ceux qui présentent une délétion 22q11.2 ou dont le système rénine-angiotensine est atteint, sont susceptibles d’avoir à la fois une cardiopathie congénitale et une prédisposition au cancer. De telles observations semblent indiquer que la carcinogenèse et la cardiopathie congénitale pourraient dans certains cas avoir des causes communes. Enfin, certaines conditions, comme la présence d’un circuit de Fontan et une cardiopathie congénitale cyanogène, pourraient avoir des effets sur plusieurs organes et finir par provoquer l’apparition d’un cancer. Il n’en demeure pas moins qu’il n’existe à l’heure actuelle aucun consensus clair quant aux stratégies appropriées de dépistage du cancer et aux modalités de surveillance des patients atteints de cardiopathie congénitale. Les médecins qui traitent des patients atteints de cardiopathie congénitale doivent être au fait de cette prédisposition potentielle et suivre à la lettre les recommandations en matière de dépistage s’appliquant à la population générale. Une approche interdisciplinaire et globale s’impose afin de combler les lacunes des connaissances dans ce domaine.
It is now expected that most individuals with congenital heart disease will survive to adulthood, including those with complex heart conditions. Maintaining lifelong medical care requires those with ...congenital heart disease to eventually transfer from pediatric to adult-oriented health care systems. Developing health care transition skills and gaining independence in managing one's own health care is imperative to this process and to ongoing medical and psychosocial success. This scientific statement reviews the recent evidence regarding transition and provides resources, components, and suggestions for development of congenital heart disease transition programs with the goals of improving patient knowledge, self-management, and self-efficacy skills to the level they are capable to eventually integrate smoothly into adult-oriented health care. Specifically, the scientific statement updates 3 sections relevant to transition programming. First, there is a review of specific factors to consider, including social determinants of health, psychosocial well-being, and neurocognitive status. The second section reviews costs of inadequate transition including the public health burden and the impairment in individual quality of life. Finally, the last section discusses considerations and suggestions for transition program design including communication platforms, a family-centered approach, and individual models. Although this scientific statement reviews recent literature surrounding transitions of care for individuals with congenital heart disease there remain significant knowledge gaps. As a field, we have yet to determine ideal timing and methods of transition, and barriers to transition and transfer remain, particularly for the underserved populations. The consequences of poor health care transition are great and garnering outcomes and information through organized, multifaceted, collaborative approaches to transition is critical to improving the lifelong care of individuals with congenital heart disease.
Changes in Hospitalization Patterns Among Patients With Congenital Heart Disease During the Transition From Adolescence to Adulthood Michelle Z. Gurvitz, Moira Inkelas, Maggie Lee, Karen Stout, Jose ...Escarce, Ruey-Kang Chang Few population-wide data exist on utilization of hospital services among adolescent and adult patients with congenital heart disease (CHD). Hospital discharge data for patients ages 12 to 44 years were analyzed and comparisons were made between those older and younger than 21 years. The younger patients were admitted to a smaller number of hospitals and were less likely to be admitted via the emergency department (ED) than those over age 21. Older patients and those without private insurance were more likely to be admitted via the ED. These findings suggest that access to specialized care may be lacking for patients with CHD during transition to adulthood and require further investigation.
Objectives This study sought to develop quality indicators (QIs) for outpatient management of adult congenital heart disease (ACHD) patients. Background There are no published QIs to promote quality ...measurement and improvement for ACHD patients. Methods Working groups of ACHD experts reviewed published data and United States, Canadian, and European guidelines to identify candidate QIs. For each QI, we specified a numerator, denominator, period of assessment, and data source. We submitted the QIs to a 9-member panel of international ACHD experts. The panel rated the QIs for validity and feasibility in 2 rounds on a scale of 1 to 9 using the RAND/University of California–Los Angeles modified-Delphi method, and final QI selection was on the basis of median scores. Results A total of 62 QIs were identified regarding appropriateness and timing of clinical management, testing, and test interpretation. Each QI was ascertainable from health records. After the first round of rating, 29 QIs were accepted, none were rejected, and 33 were equivocal; on the second round, 55 QIs were accepted. Final QIs included: 8 for atrial septal defects; 9 for aortic coarctation; 12 for Eisenmenger; 9 for Fontan; 9 for D-transposition of the great arteries; and 8 for tetralogy of Fallot. Conclusions This project resulted in development of the first set of QIs for ACHD care based on published data, guidelines, and a modified Delphi process. These QIs provide a quality of care assessment tool for 6 ACHD conditions. This rigorously designed set of QIs should facilitate measuring and improving the quality of care for this growing group of patients.
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