Changing Mortality in Congenital Heart Disease Khairy, Paul, MD, PhD; Ionescu-Ittu, Raluca, MSc; Mackie, Andrew S., MD, SM ...
Journal of the American College of Cardiology,
09/2010, Letnik:
56, Številka:
14
Journal Article
Recenzirano
Odprti dostop
Objectives This study sought to characterize temporal trends in all-cause mortality in patients with congenital heart disease (CHD). Background Historically, most deaths in patients with CHD occurred ...in early childhood. Notable advances have since been achieved that may impact on mortality trends. Methods We conducted a population-based cohort study of patients with CHD in Quebec, Canada, from July 1987 to June 2005. A total of 8,561 deaths occurred in 71,686 patients with CHD followed for 982,363 patient-years. Results The proportion of infant and childhood deaths markedly declined from 1987 to 2005, with a reduction in mortality that exceeded that of the general population. Distribution of age at death transitioned from a bimodal to unimodal, albeit skewed, pattern, more closely approximating the general population. Overall, mortality decreased by 31% (mortality rate ratio: 0.69, 95% confidence interval CI: 0.61 to 0.79) in the last (2002 to 2005) relative to the first (1987 to 1990) period of observation. Mortality rates decreased in all age groups below 65 years, with the largest reduction in infants (mortality rate ratio: 0.23, 95% CI: 0.12 to 0.47). In adults 18 to 64 years, the mortality reduction (mortality rate ratio: 0.84, 95% CI: 0.73 to 0.97) paralleled the general population. Gains in survival were mostly driven by reduced mortality in severe forms of CHD, particularly in children (mortality rate ratio: 0.33, 95% CI: 0.19 to 0.60), and were consistent across most subtypes. Conclusions Deaths in CHD have shifted away from infants and towards adults, with a steady increase in age at death and decreasing mortality.
The Canadian Cardiovascular Society (CCS) atrial fibrillation (AF) guidelines program was developed to aid clinicians in the management of these complex patients, as well as to provide direction to ...policy makers and health care systems regarding related issues. The most recent comprehensive CCS AF guidelines update was published in 2010. Since then, periodic updates were published dealing with rapidly changing areas. However, since 2010 a large number of developments had accumulated in a wide range of areas, motivating the committee to complete a thorough guideline review. The 2020 iteration of the CCS AF guidelines represents a comprehensive renewal that integrates, updates, and replaces the past decade of guidelines, recommendations, and practical tips. It is intended to be used by practicing clinicians across all disciplines who care for patients with AF. The Grading of Recommendations, Assessment, Development and Evaluations (GRADE) system was used to evaluate recommendation strength and the quality of evidence. Areas of focus include: AF classification and definitions, epidemiology, pathophysiology, clinical evaluation, screening and opportunistic AF detection, detection and management of modifiable risk factors, integrated approach to AF management, stroke prevention, arrhythmia management, sex differences, and AF in special populations. Extensive use is made of tables and figures to synthesize important material and present key concepts. This document should be an important aid for knowledge translation and a tool to help improve clinical management of this important and challenging arrhythmia.
Le programme de lignes directrices de la Société canadienne de cardiologie (SCC) en matière de fibrillation auriculaire (FA) a été élaboré pour aider les cliniciens à prendre en charge ces patients complexes, ainsi que pour orienter les décideurs politiques et les systèmes de soins de santé sur des questions connexes. La dernière édition complète des lignes directrices de la SCC en matière de FA a été publiée en 2010. Depuis lors, des mises à jour périodiques ont été publiées, traitant de domaines en évolution rapide. Cependant, en 2020, un grand nombre de développements s'y étaient ajoutés, couvrant un large éventail de domaines, ce qui a motivé le comité à créer une refonte complète des lignes directrices. L'édition 2020 des lignes directrices de la SCC en matière de FA représente un renouvellement complet qui intègre, met à jour et remplace les lignes directrices, les recommandations et les conseils pratiques des dix dernières années. Elle est destinée à être utilisée par les cliniciens praticiens de toutes les disciplines qui s'occupent de patients souffrant de FA. L'approche GRADE (Gradation des Recommandations, de l’Appréciation, du Développement et des Évaluations) a été utilisée pour évaluer la pertinence des recommandations et la qualité des résultats. Les domaines d'intérêt incluent : la classification et les définitions de la FA, son épidémiologie, sa physiopathologie, l’évaluation clinique, le dépistage de la FA, la détection et la gestion des facteurs de risque modifiables, l’approche intégrée de la gestion de la FA, la prévention des accidents vasculaires cérébraux, la gestion de l'arythmie, les différences entre les sexes et la FA dans des populations particulières. Des tableaux et figures ont été largement utilisés pour synthétiser les éléments importants et présenter les concepts clés. Ce document devrait représenter une aide importante pour l’intégration des connaissances et un outil pour aider à améliorer la gestion clinique de cette arythmie importante et difficile à traiter.
Objectives This study sought to determine the impact of steroid therapy on cardiomyopathy and mortality in patients with Duchenne muscular dystrophy (DMD). Background DMD is a debilitating X-linked ...disease that afflicts as many as 1 in 3,500 boys. Although steroids slow musculoskeletal impairment, the effects on cardiac function and mortality remain unknown. Methods We conducted a cohort study on patients with DMD treated with renin-angiotensin-aldosterone system antagonists with or without steroid therapy. Results Eighty-six patients, 9.1 ± 3.5 years of age, were followed for 11.3 ± 4.1 years. Seven of 63 patients (11%) receiving steroid therapy died compared with 10 of 23 (43%) not receiving steroid therapy (p = 0.0010). Overall survival rates at 5, 10, and 15 years of follow-up were 100%, 98.0%, and 78.6%, respectively, for patients receiving steroid therapy versus 100%, 72.1%, and 27.9%, respectively, for patients not receiving steroid therapy (log-rank p = 0.0005). In multivariate propensity-adjusted analyses, steroid use was associated with a 76% lower mortality rate (hazard ratio: 0.24; 95% confidence interval: 0.07 to 0.91; p = 0.0351). The mortality reduction was driven by fewer heart failure–related deaths (0% vs. 22%, p = 0.0010). In multivariate analyses, steroids were associated with a 62% lower rate of new-onset cardiomyopathy (hazard ratio: 0.38; 95% confidence interval: 0.16 to 0.90; p = 0.0270). Annual rates of decline in left ventricular ejection fraction (−0.43% vs. −1.09%, p = 0.0101) and shortening fraction (−0.32% vs. −0.65%, p = 0.0025) were less steep in steroid-treated patients. Consistently, the increase in left ventricular end-diastolic dimension was of lesser magnitude (+0.47 vs. +0.92 mm per year, p = 0.0105). Conclusions In patients with DMD, steroid therapy is associated with a substantial reduction in all-cause mortality and new-onset and progressive cardiomyopathy.
There is growing awareness of the long-term impact of a Fontan circulation on the associated morbidity and mortality. Comparative data on the incidence of supraventricular arrhythmia and sinus node ...dysfunction following extracardiac conduit (EC) and lateral tunnel (LT) Fontans are controversial. We performed a meta-analysis pooling all available long-term results comparing the EC and LT Fontan, with a special focus on arrhythmia.
We performed a systematic search of PubMed, Embase, and the Cochrane Library for articles reporting long-term results of Fontan comparing the EC and the LT Fontan.
Twelve studies were selected with 3,330 patients (1,729 EC, 1,601 LT). Freedom from tachyarrhythmia was significantly higher in the EC group (92% versus 83% at 15 years; p < 0.0001), while there was no difference in term of bradyarrhythmias (p = 0.7). The survival was 93% and 89% at 20 years in the EC and LT groups, respectively (p = 0.007). The risk of thromboembolic events was 2.87% patient-years in the EC group versus 0.9% in the LT group (odds ratio, 2.15; 95% confidence interval, 0.95 to 4.85; p = 0.07).
The EC Fontan confers long-term survival advantage over the LT Fontan without a higher rate of reoperations. The EC Fontan preserves the sinus node function and reduces significantly the incidence of long-term postoperative arrhythmia.
Data regarding long-term outcomes after the arterial switch operation for D-transposition of the great arteries are scarce.
A single-institution retrospective cohort study was conducted to assess ...cardiovascular outcomes after an arterial switch operation between 1983 and 1999. Patients without follow-up visits within 3 years were contacted and secondary sources of information obtained. Overall, 400 patients, 154 (38.3%) with a ventricular septal defect, 238 (59.5%) with an intact septum, and 9 (2.3%) with a Taussig-Bing anomaly, were followed for a median of 18.7 years. In perioperative survivors, overall and arrhythmia-free survival rates at 25 years were 96.7±1.8% and 96.6±0.1%, respectively. Late mortality was predominantly a result of sudden deaths and myocardial infarction. At 25 years, 75.5±2.5% remained free from surgical or catheter-based reintervention. Freedom from an adverse cardiovascular event was 92.9±1.9% at 25 years. Independent predictors were a single right coronary artery (hazard ratio, 4.58; 95% confidence interval, 1.32-15.90), P=0.0166) and postoperative heart failure (hazard ratio, 6.93; 95% confidence interval, 1.57-30.62; P=0.0107). At last follow-up, the left ventricular ejection fraction was 60.3±8.9%, 97.3% had class I symptoms, and 5.2% obstructive coronary artery disease. Peak oxygen uptake was 35.1±7.6 mL/kg/min (86.1±15.1% predicted), with a chronotropic index <80% in 34.2%. At least moderate neoaortic and pulmonary regurgitation were present in 3.4% and 6.6%, respectively, and more than mild neoaortic and pulmonary stenosis in 3.2% and 10.3%.
Long-term and arrhythmia-free survival is excellent after arterial switch operation. Although sequelae include chronotropic incompetence and neoaortic, pulmonary, and coronary artery complications, most patients maintain normal systolic function and exercise capacity.
Graphical Abstract
Graphical Abstract
Strengths of associations are semi-quantitively estimated by the number of asterisks from absent/weak (*) to moderate (**), strong (***), and very strong (***).
Atrial fibrillation (AF) recurrence after pulmonary vein isolation (PVI) is associated with PV to left atrium reconduction. Effective lesion creation necessitates adequate contact force between the ...ablation catheter and myocardium.
The purpose of this study was to study the utility of contact force-guided ablation on immediate and long-term outcomes.
Seventy-five patients with highly symptomatic paroxysmal AF underwent wide circumferential PVI using an irrigated-tip radiofrequency catheter. In 25 patients, ablation was guided by real-time contact force measurements (CF group; SmartTouch, Biosense Webster). A control group of 50 patients underwent PVI using a standard nonforce sensing catheter (standard group; ThermoCool, Biosense Webster). After PVI, all patients underwent adenosine testing to unmask dormant conduction. Patients were followed up at 3, 6, and 12 months and by transtelephonic monitoring as well.
Dormant conduction was unmasked and subsequently eliminated in 4 PV pairs (8%; 16% of patients) in the CF group and 35 PV pairs (35%; 52% of patients) in the standard group (P = .0004 per PV pair; P = .0029 per patient). The single-procedure, off-antiarrhythmic drug freedom from recurrent atrial arrhythmias at 1 year was 88% in the CF group vs 66% in the standard group (P = .047). Procedure duration and fluoroscopy time were significantly longer in the CF group (P = .0038 and P = .0001, respectively).
The use of real-time contact force guidance results in a significant reduction in the prevalence of dormant conduction with improved long-term freedom from recurrent arrhythmias. The utility of a contact force-guided approach requires evaluation in a long-term prospective randomized study.
PDF
