Organisation of paediatric echocardiography laboratories and governance of echocardiography services and training in Europe: current status, disparities, and potential solutions. A survey from the Association for European Paediatric and Congenital Cardiology (AEPC) imaging working group.

BACKGROUND: There is limited data on the organisation of paediatric echocardiography laboratories in Europe. METHODS: A structured and approved questionnaire was circulated across all 95 Association for European Paediatric and Congenital Cardiology affiliated centres. The aims were to evaluate: (1) facilities in paediatric echocardiography laboratories across Europe, (2) accredited laboratories, (3) medical/paramedical staff employed, (4) time for echocardiographic studies and reporting, and (5) training, teaching, quality improvement, and research programs. RESULTS: Respondents from forty-three centres (45%) in 22 countries completed the survey. Thirty-six centres (84%) have a dedicated paediatric echocardiography laboratory, only five (12%) of which reported they were European Association of Cardiovascular Imaging accredited. The median number of echocardiography rooms was three (range 1-12), and echocardiography machines was four (range 1-12). Only half of all the centres have dedicated imaging physiologists and/or nursing staff, while the majority (79%) have specialist imaging cardiologist(s). The median (range) duration of time for a new examination was 45 (20-60) minutes, and for repeat examination was 20 (5-30) minutes. More than half of respondents (58%) have dedicated time for reporting. An organised training program was present in most centres (78%), 44% undertake quality assurance, and 79% perform research. Guidelines for performing echocardiography were available in 32 centres (74%). CONCLUSION: Facilities, staffing levels, study times, standards in teaching/training, and quality assurance vary widely across paediatric echocardiography laboratories in Europe. Greater support and investment to facilitate improvements in staffing levels, equipment, and governance would potentially improve European paediatric echocardiography laboratories.

Differences in Aortopathy in Patients with a Bicuspid Aortic Valve with or without Aortic Coarctation.

OBJECTIVE: The combination of aortic coarctation (CoA) and bicuspid aortic valve (BAV) is assumed to be associated with a higher risk of ascending aortic dilatation and type A dissection, and current European Society of Cardiology (ESC) guidelines advise therefore to operate at a lower threshold in the presence of CoA. The aim of our study is to evaluate whether the coexistence of CoA in BAV patients is indeed associated with a higher risk of ascending aortic events (AAE). METHODS: In a retrospective study, all adult BAV patients visiting the outpatient clinic of our tertiary care center between February 2003 and February 2019 were included. The primary end point was an ascending aortic event (AAE) defined as ascending aortic dissection/rupture or preventive surgery. The secondary end points were aortic dilatation and aortic growth. RESULTS: In total, 499 BAV patients (43.7% female, age 40.3 ± 15.7 years) were included, of which 121 (24%) had a history of CoA (cBAV). An aortic event occurred in 38 (7.6%) patients at a mean age of 49.0 ± 13.6 years. In the isolated BAV group (iBAV), significantly more AAE occurred, but this was mainly driven by aortic valve dysfunction as indication for aortic surgery. There was no significant difference in the occurrence of dissection or severely dilated ascending aorta (>50mm) between the iBAV and cBAV patients (p = 0.56). The aortic diameter was significantly smaller in the cBAV group (30.3 ± 6.9 mm versus 35.7 ± 7.6 mm; p < 0.001). The median aortic diameter increase was 0.23 (interquartile range (IQR): 0.0-0.67) mm/year and was not significantly different between both groups (p = 0.74). CONCLUSION: Coexistence of CoA in BAV patients was not associated with a higher risk of aortic dissection, preventive aortic surgery, aortic dilatation, or more rapid aorta growth. This study suggests that CoA is not a risk factor in BAV patients, and the advice to operate at lower diameter should be reevaluated.

Phenotype in girls and women with Turner syndrome: Association between dysmorphic features, karyotype and cardio-aortic malformations.

INTRODUCTION: Turner syndrome (TS) is a genetic disorder characterized by the (partial) absence or a structural aberration of the second sex chromosome and is associated with a variety of phenotypes with specific physical features and cardio-aortic malformations. The objective of this study was to gain a better insight into the differences in dysmorphic features between girls and women with TS and to explore the association between these features, karyotype and cardio-aortic malformations. METHODS: This prospective study investigated 14 dysmorphic features of TS girls and women using a checklist. Three major phenotypic patterns were recognized (severe phenotype, lymphatic phenotype and skeletal phenotype). Patient data including karyotype and cardio-aortic malformations (bicuspid aortic valve (BAV) and aortic coarctation (COA)) were collected. Associations between the prevalence of dysmorphic features, karyotype and cardio-aortic malformations were analysed using chi2-test and odds ratios. RESULTS: A total of 202 patients (84 girls and 118 women) were analysed prospectively. Differences in prevalence of dysmorphic features were found between girls and women. A strong association was found between monosomy 45,X and the phenotypic patterns. Furthermore, an association was found between COA and lymphatic phenotype, but no association was found between karyotype and cardio-aortic malformations. CONCLUSION: This study uncovered a difference in dysmorphic features between girls and women. Monosomy 45,X is associated with a more severe phenotype, lymphatic phenotype and skeletal phenotype. All patients with TS should be screened for cardio-aortic malformations, because in contrast to previous reports, karyotype and cardio-aortic malformations showed no significant association.

Aortic Diameter Growth in Children With a Bicuspid Aortic Valve.

Knowledge of aortic growth in patients with bicuspid aortic valve (BAV) is essential to identify patients at risk for dissection, but data on children remain unclear. We retrospectively evaluated the aortic diameters of all pediatric BAV patients, identified through an echocardiographic database (2005 to 2013). Medical records were reviewed and aortic diameters re-measured on echocardiographic images at diagnosis and if available on variable mid- and endpoints follow-up. Dilatation (z-score >2) was based on 2 different z-score equation methods (Gautier/Campens). In 234 of the total 250 BAV patients, aortic diameters were analyzed; median age was 6.1 years (interquartile range 1.7 to 10), of which 63% were male. Aortic coarctation was present in 81 (36%) patients, 23% had a ventricular septal defect. BAV morphology according to Sievers was as follows: type 0 in 128 patients (55%), type 1 in 96 (41%), and type 2 in 10 (4%). Ascending aortic (AA) dilatation was present in 24% (Gautier) and 36% (Campens) at inclusion. Median follow-up was 4.7 years. The AA was the only location where mean z-scores progressed significantly with age: 0.06 (Gautier) and 0.09 (Campens) units per year between ages 5 and 15 years. Associations for higher AA z-scores at older age were an initial z-score >2 (p <0.001) and aortic valve stenosis (p <0.05). Neither dissection nor preventive aortic surgery occurred. In conclusion, only the AA seems at risk for complication, although no aortic complications occurred in this pediatric BAV cohort. BAV morphology seems associated with larger AA z-scores and valvular dysfunction.

Does functional health status predict health-related quality of life in children after Fontan operation?

PURPOSE: It is important to identify those children with a Fontan circulation who are at risk for impaired health-related quality of life. We aimed to determine the predictive value of functional health status - medical history and present medical status - on both physical and psychosocial domains of health-related quality of life, as reported by patients themselves and their parents. METHODS: We carried out a prospective cross-sectional multi-centre study in Fontan patients aged between 8 and 15, who had undergone staged completion of total cavopulmonary connection according to a current technique before the age of 7 years. Functional health status was assessed as medical history - that is, age at Fontan, type of Fontan, ventricular dominance, and number of cardiac surgical procedures - and present medical status - assessed with magnetic resonance imaging, exercise testing, and rhythm assessment. Health-related quality of life was assessed with The TNO/AZL Child Questionnaire Child Form and Parent Form. RESULTS: In multivariate prediction models, several medical history variables, such as more operations post-Fontan completion, lower age at Fontan completion, and dominant right ventricle, and present medical status variables, such as smaller end-diastolic volume, a higher score for ventilatory efficiency, and the presence of sinus node dysfunction, predicted worse outcomes on several parent-reported and self-reported physical as well as psychosocial health-related quality of life domains. CONCLUSIONS: Medical history and worse present medical status not only predicted worse physical parent-reported and self-reported health-related quality of life but also worse psychosocial health-related quality of life and subjective cognitive functioning. These findings will help in identifying patients who are at risk for developing impaired health-related quality of life.

Does exercise training improve cardiopulmonary fitness and daily physical activity in children and young adults with corrected tetralogy of Fallot or Fontan circulation? A randomized controlled trial.

BACKGROUND: Many patients with congenital heart disease do not meet current public health guidelines to participate in moderate-to-vigorous physical activity for ≥60 minutes per day. They are less fit than their healthy peers. We hypothesized that exercise training would increase cardiopulmonary fitness and daily physical activity in these patients. We therefore assessed effects of an exercise training program on cardiopulmonary fitness and daily physical activity in patients with corrected tetralogy of Fallot (ToF) or Fontan circulation. METHODS: In a multicenter prospective controlled trial, patients with ToF or Fontan circulation (age 10-25 years) were randomized, 56 patients to the exercise group and 37 to the control group. The exercise group participated in a 12-week standardized aerobic exercise training program. The control group continued lifestyle as usual. Cardiopulmonary exercise testing and activity measurements were performed before and after 12 weeks. RESULTS: Peak oxygen uptake increased in the exercise group by 5.0% (1.7 ± 4.2 mL/kg per minute; P = .011) but not in the control group (0.9 ± 5.2 mL/kg per minute; P = not significant). Workload increased significantly in the exercise group compared with the control group (6.9 ± 11.8 vs 0.8 ± 13.9 W; P = .047). Subgroup analysis showed a significant increase in pre-to-post peak oxygen uptake in the exercise group of ToF patients but not in the exercise group of Fontan patients. Percentage of measured time spent in moderate-to-vigorous activity at baseline was 13.6% ± 8.6%, which did not significantly change after training. CONCLUSIONS: Aerobic exercise training improved cardiopulmonary fitness in patients with ToF but not in patients with Fontan circulation. Exercise training did not change daily physical activity.

Computational fluid dynamics in Fontan patients to evaluate power loss during simulated exercise.

OBJECTIVE: Exercise intolerance is common in total cavopulmonary connection (TCPC) patients. It has been suggested that power loss (Ploss) inside the TCPC plays a role in reduced exercise performance. Our objective is to establish the role of Ploss inside the TCPC during increased flow, simulating exercise in a patient-specific way. METHODS: Cardiac MRI (CMR) was used to obtain flow rates from the caval veins during rest and increased flow, simulating exercise with dobutamine. A 3D reconstruction of the TCPC was created using CMR data. Computational fluid dynamics (CFD) simulations were performed to calculate Ploss inside the TCPC structure for rest and stress conditions. To reflect the flow distribution during exercise, a condition where inferior caval vein (IVC) flow was increased twofold compared with rest was added. 29 TCPC patients (15 intra-atrial lateral tunnel (ILT) and 14 extracardiac conduit (ECC)) were included. RESULTS: Mean Ploss at rest was 1.36 ± 0.94 (ILT) and 3.20 ± 1.26 (ECC) mW/m(2) (p<0.001), 2.84 ±1.95 (ILT) and 8.41 ± 3.77 (ECC) mW/m(2) (p<0.001) during dobutamine and 5.21 ± 3.50 (ILT) and 15.28 ± 8.30 (ECC) mW/m(2) (p=0.001) with twofold IVC flow. The correlation between cardiac index and Ploss was exponential (ILT: R(2)=0.811, p<0.001; ECC: R(2)=0.690, p<0.001). CONCLUSIONS: Ploss inside the TCPC structure is limited but increases with simulated exercise. This relates to the anatomy of TCPC and the surgical technique used. In all flow conditions, ILT patients have lower Ploss than ECC patients. We did not find a relationship between Ploss and exercise capacity.

Aerobic exercise influences quality of life of children and youngsters with congenital heart disease: a randomized controlled trial.

PURPOSE: To evaluate effects of an exercise program on health-related quality of life (HRQoL) in children and adolescents with tetralogy of Fallot (ToF) or a Fontan circulation. METHODS: Stratified, randomized, controlled intervention study conducted in five participating centers of pediatric cardiology in The Netherlands. In total, 93 patients, aged 10-25 years, with surgical repair for tetralogy of Fallot or with a Fontan circulation for single-ventricle physiology were included. They were randomly allocated with a ratio of 2:1 to: (1) a 12-week period with an exercise program for 3 times per week or (2) to a control group. Randomization was stratified by age, gender, and cardiac diagnosis. At baseline and follow-up after 12 weeks, all participants completed Web-based age-appropriate HRQoL questionnaires. Primary analyses involved change in HRQoL during follow-up. Secondary analyses concerned influence of cardiac diagnosis and comparison with normative data. RESULTS: Forty-eight (86%) and 32 (86%) patients in the exercise-group and control-group respectively completed all questionnaires at baseline and follow-up. Compared with the control-group, children, aged 10-15 years, in the exercise-group improved significantly on self-reported cognitive functioning, p < .05, r = .30, and parent-reported social functioning, p < .05, r = .30. Youngsters aged 16-25 years did not change their HRQoL. Cardiac diagnosis had no influence on pre/post changes. Children and youngsters in this study reported comparable or better HRQoL than norm groups. CONCLUSIONS: Participation in an exercise program improved HRQoL of children with ToF or a Fontan circulation, especially in those with low baseline QoL.

Exercise capacity in children after total cavopulmonary connection: lateral tunnel versus extracardiac conduit technique.

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.

Vascular function in children with repaired tetralogy of Fallot.

We compared the endothelial function and vascular wall characteristics of 11 children with tetralogy of Fallot (TOF) (age 13 +/- 3 years) with the characteristics of 17 age-matched peers (12 +/- 2 years). Echocardiographic Doppler measurements were performed under standardized conditions to assess (1) the carotid and femoral artery diameter and intima-media thickness, (2) brachial artery endothelial function using flow-mediated dilation, and (3) central and peripheral compliance using pulsewave velocity. In addition, the physical activity level was assessed using a validated questionnaire. We found that the physical activity level of the children with TOF was lower than that of the controls, but the difference did not reach statistical significance (4.5 vs 5.9 h/wk, p = 0.087). A significantly larger femoral artery intima-media thickness was observed in those with TOF, and the carotid and brachial artery diameter and intima-media thickness were comparable between groups. The children with TOF demonstrated a significantly lower brachial artery flow-mediated dilation than that of the controls. The central and peripheral compliance did not differ between the 2 groups. In conclusion, children with TOF demonstrated an impaired brachial artery endothelial function and increased intima-media thickness of the femoral artery compared to their healthy peers. In conclusion, our findings have, therefore, indicated that children with TOF, already at a young age, have changes in vascular function and structure.

Exercise performance and activity level in children with transposition of the great arteries treated by the arterial switch operation.

The exercise capacity of children after arterial switch for transposition of the great arteries (TGA) is known to be at the lower limit of normal. We aimed to ascertain whether this results from compromised hemodynamics or deconditioning. A total of 17 children with TGA (12 male and 5 female children; age 12.1 + or - 2.0 years) treated with the arterial switch operation were compared with 20 age-matched controls (13 male and 7 female children; age 12.8 + or - 2.4 years) regarding their peak exercise capacity, peak workload, and peak heart rate, as assessed by cycle ergometry. The children's physical activity level was monitored for a 7-day period using a pedometer and diary, and a questionnaire was used to assess physical activity participation and overprotection. The results demonstrated that TGA children showed a significantly reduced peak exercise capacity (47.4 + or - 6.4 vs 41.1 + or - 6.6 ml/kg/min; p <0.05), maximal workload (3.7 + or - 0.5 vs 3.1 + or - 0.6 W/kg; p <0.01), and maximal heart rate (189 + or - 9 vs 180 + or - 14 beats/min; p <0.05) compared to the controls. No significant differences were found in the physical activity pattern or overprotection. In conclusion, given the comparable physical activity level, but reduced exercise capacity in the TGA children, these children most likely fall short in their exercise performance because of restrictive hemodynamics rather than deconditioning from reduced daily life activity.

Exercise capacity and participation of children with a ventricular septal defect.

Existing data on exercise performance in children with a ventricular septal defect (VSD) are scarce and inconclusive. We aimed to elucidate whether and why exercise capacity and physical activity level are decreased in children with VSD. Children 9 to 17 years of age with a surgically (operated, n = 13) or conservatively (unoperated, n = 14) managed VSD were compared with healthy peers (controls, n = 15) regarding (1) anthropometric and spirometric measurements; (2) cycle ergometric performance in terms of peak oxygen uptake, peak heart rate, and peak workload; and (3) mean daily energy expenditure. All subjects completed a questionnaire on physical activity participation, overprotection, and self-perceived fitness and health. Anthropometric variables were comparable among groups, whereas lung function at rest was mildly decreased in the 2 VSD subgroups. Exercise testing succeeded in all 42 subjects; valid results were obtained in 39. Weight- and body fat-adjusted peak oxygen uptake, peak workload, and energy expenditure were not significantly different among groups. Peak heart rate emerged as significantly lower in operated subjects, which was not attributable to poorer effort. Physical fitness and general health were rated comparably among groups, whereas participation in sports was markedly lower in children with VSD. Discouragement from engaging in (strenuous) exercise occurred equally infrequently in children with VSD and controls. In conclusion, children with patent or surgically closed VSDs have a normal exercise capacity, despite a mild chronotropic limitation in the latter. Previous cardiac surgery did not influence these children's exercise performance. These children consider themselves healthy, they exhibit a normal habitual physical activity level, and they are not withheld from (strenuous) exercise.