Congenital heart disease (CHD) accounts for nearly one-third of all major congenital anomalies. CHD birth prevalence worldwide and over time is suggested to vary; however, a complete overview is ...missing. This systematic review included 114 papers, comprising a total study population of 24,091,867 live births with CHD identified in 164,396 individuals. Birth prevalence of total CHD and the 8 most common subtypes were pooled in 5-year time periods since 1930 and in continent and income groups since 1970 using the inverse variance method. Reported total CHD birth prevalence increased substantially over time, from 0.6 per 1,000 live births (95% confidence interval CI: 0.4 to 0.8) in 1930 to 1934 to 9.1 per 1,000 live births (95% CI: 9.0 to 9.2) after 1995. Over the last 15 years, stabilization occurred, corresponding to 1.35 million newborns with CHD every year. Significant geographical differences were found. Asia reported the highest CHD birth prevalence, with 9.3 per 1,000 live births (95% CI: 8.9 to 9.7), with relatively more pulmonary outflow obstructions and fewer left ventricular outflow tract obstructions. Reported total CHD birth prevalence in Europe was significantly higher than in North America (8.2 per 1,000 live births 95% CI: 8.1 to 8.3 vs. 6.9 per 1,000 live births 95% CI: 6.7 to 7.1; p < 0.001). Access to health care is still limited in many parts of the world, as are diagnostic facilities, probably accounting for differences in reported birth prevalence between high- and low-income countries. Observed differences may also be of genetic, environmental, socioeconomical, or ethnic origin, and there needs to be further investigation to tailor the management of this global health problem.
Abstract Objective Despite an increasing interest in pediatric aortic valve repair, aortic valve replacement in children may be unavoidable. The evidence on outcome after pediatric aortic valve ...replacement is limited and usually reported in small case series. This systematic review and meta-analysis aims to provide an overview of reported outcome of pediatric patients after aortic valve replacement. Methods A systematic literature search for publications reporting outcome after pediatric aortic valve replacement published between January 1990 and May 2015 was conducted. Studies written in English with a study size of more than 30 patients were included. Results Thirty-four publications reporting on 42 cohorts were included in this review: 26 concerning the Ross procedure (n = 2409), 13 concerning mechanical prosthesis aortic valve replacement (n = 696), and 3 concerning homograft aortic valve replacement (n = 224). There were no studies on bioprostheses that met our inclusion criteria. The pooled mean patient age was 9.4 years, 12.8 years, and 8.9 years for Ross, mechanical prosthesis, and homograft recipients, respectively. Pooled mean follow-up was 6.6 years. The Ross procedure was associated with lower early (4.20%; 95% confidence interval CI, 3.37-5.22 vs 7.34%; 95% CI, 5.21-10.34 vs 12.82%; 95% CI, 8.91-18.46) and late mortality (0.64%/y; 95% CI, 0.49-0.84 vs 1.23%/y; 95% CI, 0.85-1.79 vs 1.59%/y; 95% CI, 1.03-2.46) compared with mechanical prosthesis aortic valve replacement and homograft aortic valve replacement, respectively. No significantly different aortic valve reoperation rates were observed between the Ross procedure and mechanical prosthesis aortic valve replacement (1.60%/y; 95% CI, 1.27-2.02 vs 1.07%/y; 95% CI, 0.68-1.68, respectively), whereas homograft aortic valve replacement was associated with significantly higher aortic valve reoperation rates (5.44%/y; 95% CI, 4.24-6.98). The Ross procedure–associated right ventricular outflow tract reoperation rate was 1.91% per year (95% CI, 1.50-2.44). Conclusions This systematic review illustrates that all currently available aortic valve substitutes are associated with suboptimal results in children, reflecting the urgent need for reliable and durable repair techniques and innovative replacement solutions for this challenging group of patients.
Tetralogy of Fallot (ToF) is the most common type of cyanotic congenital heart disease. Since the first surgical repair in 1954, treatment has continuously improved. The treatment strategies ...currently used in the treatment of ToF result in excellent long-term survival (30 year survival ranges from 68.5% to 90.5%). However, residual problems such as right ventricular outflow tract obstruction, pulmonary regurgitation, and (ventricular) arrhythmia are common and often require re-interventions. Right ventricular dysfunction can be seen following longstanding pulmonary regurgitation and/or stenosis. Performing pulmonary valve replacement or relief of pulmonary stenosis before irreversible right ventricular dysfunction occurs is important, but determining the optimal timing of pulmonary valve replacement is challenging for several reasons. The biological mechanisms underlying dysfunction of the right ventricle as seen in longstanding pulmonary regurgitation are poorly understood. Different methods of assessing the right ventricle are used to predict impending dysfunction. The atrioventricular, ventriculo-arterial and interventricular interactions of the right ventricle play an important role in right ventricle performance, but are not fully elucidated. In this review we present a brief overview of the history of ToF, describe the treatment strategies currently used, and outline the long-term survival, residual lesions, and re-interventions following repair. We discuss important remaining challenges and present the current state of the art regarding these challenges.
Abstract
Aims
We examined the associations of pericardial adipose tissue with cardiac structures and cardiovascular risk factors in children.
Methods and results
We performed a cross-sectional ...analysis in a population-based cohort study among 2892 children aged 10 years (2404 normal weight and 488 overweight/obese). Pericardial adipose tissue mass was estimated by magnetic resonance imaging (MRI) and indexed on height3. Left ventricular mass (LVM) and left ventricular mass-to-volume ratio (LMVR) were estimated by cardiac MRI. Cardiovascular risk factors included android adipose tissue percentage obtained by Dual-energy X-ray absorptiometry, blood pressure and glucose, insulin, cholesterol, and triglycerides concentrations. Adverse outcomes were defined as values above the 75 percentile. Median pericardial adipose tissue index was 3.6 (95% range 1.6–7.1) among normal weight and 4.7 (95% range 2.0–8.9) among overweight children. A one standard deviation (1 SD) higher pericardial adipose tissue index was associated with higher LMVR 0.06 standard deviation scores, 95% confidence interval (CI) 0.02–0.09, increased odds of high android adipose tissue odd ratio (OR) 2.08, 95% CI 1.89–2.29, high insulin concentrations (OR 1.17, 95% CI 1.06–1.30), an atherogenic lipid profile (OR 1.22, 95% CI 1.11–1.33), and clustering of cardiovascular risk factors (OR 1.56, 95% CI 1.36–1.79). Pericardial adipose tissue index was not associated with LVM, blood pressure, and glucose concentrations. The associations showed largely the same directions but tended to be weaker among normal weight than among overweight children.
Conclusion
Pericardial adipose tissue is associated with cardiac adaptations and cardiovascular risk factors already in childhood in both normal weight and overweight children.
The method used to delineate the boundary of the right ventricle (RV), relative to the trabeculations and papillary muscles in cardiovascular magnetic resonance (CMR) ventricular volume analysis, may ...matter more when these structures are hypertrophied than in individuals with normal cardiovascular anatomy. This study aimed to compare two methods of cavity delineation in patients with systemic RV.
Twenty-nine patients (mean age 34.7 +/- 12.4 years) with a systemic RV (12 with congenitally corrected transposition of the great arteries (ccTGA) and 17 with atrially switched (TGA) underwent CMR. We compared measurements of systemic RV volumes and function using two analysis protocols. The RV trabeculations and papillary muscles were either included in the calculated blood volume, the boundary drawn immediately within the apparently compacted myocardial layer, or they were manually outlined and excluded. RV stroke volume (SV) calculated using each method was compared with corresponding left ventricular (LV) SV. Additionally, we compared the differences in analysis time, and in intra- and inter-observer variability between the two methods. Paired samples t-test was used to test for differences in volumes, function and analysis time between the two methods. Differences in intra- and inter-observer reproducibility were tested using an extension of the Bland-Altman method.
The inclusion of trabeculations and papillary muscles in the ventricular volume resulted in higher values for systemic RV end diastolic volume (mean difference 28.7 +/- 10.6 ml, p < 0.001) and for end systolic volume (mean difference 31.0 +/- 11.5 ml, p < 0.001). Values for ejection fraction were significantly lower (mean difference -7.4 +/- 3.9%, p < 0.001) if structures were included. LV SV did not differ significantly from RV SV for both analysis methods (p = NS). Including structures resulted in shorter analysis time (p < 0.001), and showed better inter-observer reproducibility for ejection fraction (p < 0.01).
The choice of method for systemic RV cavity delineation significantly affected volume measurements, given the CMR acquisition and analysis systems used. We recommend delineation outside the trabeculations for routine clinical measurements of systemic RV volumes as this approach took less time and gave more reproducible measurements.
Objectives In patients with univentricular heart disease, the total cavopulmonary connection (TCPC) is the preferred treatment. TCPC can be performed using the intra-atrial lateral tunnel (ILT) or ...extracardiac conduit (ECC) technique. The purpose of the present study was to evaluate exercise capacity in contemporary TCPC patients and compare the results between the 2 techniques. Methods A total of 101 TCPC patients (ILT, n = 42; ECC, n = 59; age, 12.2 ± 2.6 years; age at TCPC completion, 3.2 ± 1.1 years) underwent cardiopulmonary exercise testing. The patients were recruited prospectively from 5 tertiary referral centers. Results For the entire group, the mean peak oxygen uptake was 74% ± 14%, peak heart rate was 90% ± 8%, peak workload was 62% ± 13%, and slope of ventilation versus carbon dioxide elimination (VE/VCO2 slope) was 127% ± 30% of the predicted value. For the ILT and ECC groups, patient age, age at TCPC completion, body surface area, peak workload, and peak heart rate were comparable. The percentage of predicted peak oxygen uptake was lower in the ILT group (70% ± 12% vs 77% ± 15%; P = .040), and the percentage of predicted VE/VCO2 slope was greater in the ILT group (123% ± 36% vs 108% ± 14%; P = .015). In a subgroup analysis that excluded ILT patients with baffle leak, these differences were not statistically significant. Conclusions Common exercise parameters were impaired in contemporary Fontan patients. Chronotropic incompetence was uncommon. The peak oxygen uptake and VE/VCO2 slope were less favorable in ILT patients, likely related to baffle leaks in some ILT patients. These results have shown that a reduced exercise capacity in Fontan patients remains an important issue in contemporary cohorts. The ECC had a more favorable exercise outcome at medium-term follow-up.
Children and adults with repaired tetralogy of Fallot (rTOF) have an impaired exercise capacity, a less active lifestyle and an increased long-term risk of adverse outcomes compared with healthy ...peers. This study aimed to summarise the current evidence for the effectiveness and safety of exercise training interventions in patients with rTOF.
PubMed/MEDLINE, EMBASE, the Cochrane Central Register of Controlled Trials (CENTRAL), Web of Science, Scopus and reference lists of relevant articles were searched for prospective studies published by November 2021. Random-effects meta-analysis and descriptive synthesis were performed to assess the effectiveness and safety of exercise training in patients with rTOF.
Of the 9677 citations identified, 12 articles were included that reported on 10 unique studies and covered 208 patients with rTOF (range of mean/median age: 7.4-43.3 years). All studies implemented 2 to 7 aerobic or respiratory training sessions per week with durations ranging from 6 to 26 weeks. Meta-analysis of the included randomised controlled trials showed that exercise training was associated with a significant improvement in peak VO
(pooled mean difference: +3.1 mL/min/kg; 95% CI: 0.76 to 5.36 mL/min/kg, p=0.019). Cardiac imaging studies revealed no subclinical adverse remodelling after the exercise interventions. No serious adverse events including arrhythmias were reported in these studies.
Current evidence suggests that exercise training can improve exercise capacity in patients with rTOF with a low risk for adverse events. Exercise prescription may be a safe and effective tool to help improving outcomes in patients with rTOF.
CRD42021292809.
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