Preeclampsia and neonatal outcomes in pregnancies with maternal congenital heart disease: A nationwide cohort study from Norway.

INTRODUCTION: The prevalence of congenital heart disease (CHD) among women of reproductive age is rising. We aimed to investigate the risk of preeclampsia and adverse neonatal outcomes in pregnancies of mothers with CHD compared to pregnancies of mothers without heart disease. MATERIAL AND METHODS: In a nationwide cohort of pregnancies in Norway 1994-2014, we retrieved information on maternal heart disease, the course of pregnancy, and neonatal outcomes from national registries. Comparing pregnancies with maternal CHD to pregnancies without maternal heart disease, we used Cox regression to estimate the adjusted hazard ratio (aHR) for preeclampsia and log-binomial regression to estimate the adjusted risk ratio (aRR) for adverse neonatal outcomes. The estimates were adjusted for maternal age and year of childbirth and presented with 95% confidence intervals (CIs). RESULTS: Among 1 218 452 pregnancies, 2425 had mild maternal CHD, and 603 had moderate/severe CHD. Compared to pregnancies without maternal heart disease, the risk of preeclampsia was increased in pregnancies with mild and moderate/severe maternal CHD (aHR1.37, 95% CI 1.14-1.65 and aHR 1.62, 95% CI 1.13-2.32). The risk of preterm birth was increased in pregnancies with mild maternal CHD (aRR 1.33, 95% CI 1.15-1.54) and further increased with moderate/severe CHD (aRR 2.49, 95% CI 2.03-3.07). Maternal CHD was associated with elevated risks of both spontaneous and iatrogenic preterm birth. The risk of infants small-for-gestational-age was slightly increased with mild maternal CHD (aRR 1.12, 95% CI 1.00-1.26) and increased with moderate/severe CHD (aRR 1.63, 95% CI 1.36-1.95). The prevalence of stillbirth was 3.9 per 1000 pregnancies without maternal heart disease, 5.6 per 1000 with mild maternal CHD, and 6.8 per 1000 with moderate/severe maternal CHD. Still, there were too few cases to report a significant difference. There were no maternal deaths in women with CHD. CONCLUSIONS: Moderate/severe maternal CHD in pregnancy was associated with increased risks of preeclampsia, preterm birth, and infants small-for-gestational-age. Mild maternal CHD was associated with less increased risks. For women with moderate/severe CHD, their risk of preeclampsia and adverse neonatal outcomes should be evaluated together with their cardiac risk in pregnancy, and follow-up in pregnancy should be ascertained.

Increasing region of interest width reduces neonatal circumferential strain.

OBJECTIVE: There is growing interest in speckle tracking echocardiography-derived strain as a measure of left ventricular function in neonates. However, knowledge gaps remain regarding the effect of image acquisition and processing parameters on circumferential strain measurements. The aim of this study was to evaluate the effect of using different region of interest (ROI) widths on speckle tracking derived circumferential strain in healthy neonates. METHODS: Thirty healthy-term-born neonates were examined with speckle-tracking echocardiography in the short-axis view. Circumferential strain values were acquired and compared using two different ROI widths. Furthermore, strain values in the different vendor-defined wall layers were also compared. RESULTS: Increasing ROI width led to a decrease in global circumferential strain (GCS) in the midwall and epicardial layers, the respective decreases in strain being -23.4 ± .6% to -22.0 ± 1.1%, p < .0001 and 18.5 ± 1.7% to -15.6 ± 2.0%, p < .0001. Segmental analyses were consistent with these results, apart from two segments in the midwall. There was no statistically significant effect on strain for the endocardial layer. A gradient was seen where strain increased from the epicardial to endocardial layers. CONCLUSION: Increasing ROI width led to a decrease in GCS in the midwall and epicardium. There is an increase in circumferential strain when moving from the epicardial toward the endocardial layer. Clinicians wishing to implement circumferential strain into their practice should consider ROI width variation as a potential confounder in their measurements.

Systolic myocardial function measured by echocardiographic speckle-tracking and peak oxygen consumption in pediatric childhood cancer survivors-a PACCS study.

Background: Cancer therapy-related cardiotoxicity is a major cause of cardiovascular morbidity in childhood cancer survivors. The aims of this study were to investigate systolic myocardial function and its association to cardiorespiratory fitness in pediatric childhood cancer survivors. Methods: In this sub-study of the international study "Physical Activity and fitness in Childhood Cancer Survivors" (PACCS), echocardiographic measures of left ventricular global longitudinal strain (LV-GLS) and right ventricular longitudinal strain (RV-LS) were measured in 128 childhood cancer survivors aged 9-18 years and in 23 age- and sex-matched controls. Cardiorespiratory fitness was measured as peak oxygen consumption achieved on treadmill and correlated to myocardial function. Results: Mean LV-GLS was reduced in the childhood cancer survivors compared to the controls, -19.7% [95% confidence interval (CI) -20.1% to -19.3%] vs. -21.3% (95% CI: -22.2% to -20.3%) (p = 0.004), however, mainly within normal range. Only 13% of the childhood cancer survivors had reduced LV longitudinal strain z-score. Mean RV-LS was similar in the childhood cancer survivors and the controls, -23.2% (95% CI: -23.7% to -22.6%) vs. -23.3% (95% CI: -24.6% to -22.0%) (p = 0.8). In the childhood cancer survivors, lower myocardial function was associated with lower peak oxygen consumption [correlation coefficient (r) = -0.3 for LV-GLS]. Higher doses of anthracyclines (r = 0.5 for LV-GLS and 0.2 for RV-LS) and increasing time after treatment (r = 0.3 for LV-GLS and 0.2 for RV-LS) were associated with lower myocardial function. Conclusions: Left ventricular function, but not right ventricular function, was reduced in pediatric childhood cancer survivors compared to controls, and a lower left ventricular myocardial function was associated with lower peak oxygen consumption. Furthermore, higher anthracycline doses and increasing time after treatment were associated with lower myocardial function, implying that long-term follow-up is important in this population at risk.

Myocardial function including estimates of myocardial work in young adults born very preterm or with extremely low birthweight - a cohort study.

BACKGROUND: Preterm birth and low birthweight have been associated with increased risk of heart failure and cardiovascular disease in young adulthood. However, results from clinical studies of myocardial function are not consistent. Echocardiographic strain analyses allow detection of early stages of cardiac dysfunction, and non-invasive estimates of myocardial work can provide additional information on cardiac function. We aimed to evaluate left ventricular (LV) myocardial function including measures of myocardial work in young adults born very preterm (gestational age < 29 weeks) or with extremely low birthweight (< 1000 g) (PB/ELBW), compared with term-born age- and sex matched controls. METHODS: 63 PB/ELBW and 64 controls born in Norway in the periods 1982-1985, 1991-1992, and 1999-2000 were examined with echocardiography. LV ejection fraction (EF) and LV global longitudinal strain (GLS) were measured. Myocardial work was estimated from LV pressure-strain loops after determination of GLS and construction of a LV pressure curve. Diastolic function was evaluated by determination of the presence or absence of elevated LV filling pressure, including measures of left atrial longitudinal strain. RESULTS: The PB/ELBW with mean birthweight 945 (standard deviation (SD) 217) grams, mean gestational age 27 (SD 2) weeks, and mean age 27 (SD 6) years had LV systolic function mainly within normal range. Only 6% had EF < 50% or impaired GLS >-16%, but 22% had borderline impaired GLS between - 16% and - 18%. Mean GLS in PB/ELBW (-19.4% (95% confidence interval (CI) -20.0, -18.9)) was impaired compared to controls (-20.6% (95% CI -21.1, -20.1)), p = 0.003. Lower birthweight was associated to more impaired GLS (Pearson correlation coefficient - 0.2). Means of EF, measures of diastolic function including left atrial reservoir strain, global constructive and wasted work, global work index and global work efficiency was similar in PB/ELBW and controls. CONCLUSION: The young adults born very preterm or with extremely low birthweight had impaired LV-GLS compared to controls, although systolic function mainly within normal range. Lower birthweight was associated with more impaired LV-GLS. These findings could indicate an elevated lifetime risk of developing heart failure in preterm born individuals. Measures of diastolic function and myocardial work were similar compared to controls.

Cause-Specific Mortality in Patients During Long-Term Follow-Up After Atrial Switch for Transposition of the Great Arteries.

Background Little is known about the cause of death (CoD) in patients with transposition of the great arteries palliated with a Mustard or Senning procedure. The aim was to describe the CoD for patients with the Mustard and Senning procedure during short- (<10 years), mid- (10-20 years), and long-term (>20 years) follow-up after the operation. Methods and Results This is a retrospective, descriptive multicenter cohort study including all Nordic patients (Denmark, Finland, Norway, and Sweden) who underwent a Mustard or Senning procedure between 1967 and 2003. Patients who died within 30 days after the index operation were excluded. Among 968 patients with Mustard/Senning palliated transposition of the great arteries, 814 patients were eligible for the study, with a mean follow-up of 33.6 years. The estimated risk of all-cause mortality reached 36.0% after 43 years of follow-up, and the risk of death was highest among male patients as compared with female patients (P=0.004). The most common CoD was sudden cardiac death (SCD), followed by heart failure/heart transplantation accounting for 29% and 27%, respectively. During short-, mid-, and long-term follow-up, there was a change in CoD with SCD accounting for 23.7%, 46.6%, and 19.0% (P=0.002) and heart failure/heart transplantation 18.6%, 22.4%, and 46.6% (P=0.0005), respectively. Conclusions Among patients corrected with Mustard or Senning transposition of the great arteries, the most common CoD is SCD followed by heart failure/heart transplantation. The CoD changes as the patients age, with SCD as the most common cause in adolescence and heart failure as the dominant cause in adulthood. Furthermore, the risk of all-cause mortality, SCD, and death attributable to heart failure or heart transplantation was increased in men >10 years after the Mustard/Senning operation.

Physical and mental health in young adults with heart disease - a national survey of Norwegian university students.

BACKGROUND: Young adults with heart disease constitute a growing group with the risk of cognitive and physical impairment. The knowledge of their academic performance and mental and physical health is, however, scant. This study aimed to compare young adults with CHDs or arrhythmia with their peers. METHODS: Information on physical health (Somatic Symptom Scale-8), mental health problems (Hopkins Symptoms Checklist-25), quality of life (Satisfaction With Life Scale), physical activity, and academic performance was collected online in a national cross-sectional survey in Norway among students in higher education (the SHoT2018 study). RESULTS: Among 50,054 students, 172 (0.34%) reported CHD and 132 (0.26%) arrhythmias. Students reporting arrhythmias scored significantly higher than the control group on somatic symptoms (OR = 2.3 (95% CI: 1.62-3.27)), anxiety (OR = 1.60 (1.08-2.37)), depression (OR = 1.49 (1.05-2.11)), self-harm, and suicide attempt (OR = 2.72 (1.56-4.75)), and lower quality of life (OR 1.64 (1.16-2.32)) and more loneliness (OR = 1.99 (1.28-3.10)) compared to participants without heart disease. Participants with CHD reported an increased somatic symptom burden (OR = 1.58 (1.16-2.16)). Despite a tendency to a higher score, this group did not differ significantly from the control group on anxiety or depression, quality of life, or loneliness. However, the risk of self-harm thoughts and suicidality was significantly increased (OR for suicide attempt 2.22 (1.3-3.77)). There was no difference between the groups on academic performance. CONCLUSIONS: Although Norwegian students with heart disease reported more somatic symptoms, their academic progress was not reduced compared to students without heart disease. Students with CHD or arrhythmias showed an increased risk of self-harm thoughts and suicidality.

The influence of region of interest width in fetal 2D-speckle tracking echocardiography late in pregnancy.

Speckle tracking echocardiography is a promising method for assessment of myocardial function in fetal and neonatal hearts, but further studies are necessary to validate and optimize the settings for use in fetal cardiology. Previous studies have shown that the definition of the region of interest (ROI) affects strain values in adults. The aim of this study was to investigate how different widths of ROI influences measurements of four-chamber longitudinal systolic strain in fetuses late in pregnancy. Thirty-one singleton, healthy fetuses born to healthy mothers underwent an echocardiographic examination during gestational week 37. Speckle tracking was performed with two different settings for ROI width; the narrowest and second most narrow, provided both widths were assessed as suitable for the myocardial wall thickness of the fetus. We found an inverse correlation between the ROI width and the strain values. Four-chamber longitudinal strain changed from - 20.7 ± 3.6% to - 18.0 ± 4.4% (p < 0.001) with increasing ROI width. Further, strain decreased from the endocardium to the epicardium with multilayer measurements. Different widths of ROI influenced the strain measurements significantly in the fetal heart, comparable to what has been reported in adults. A standardization of the ROI setting could improve the interpretation, and reduce variability in fetal strain measurements.

Vascular Endothelial Function Assessed by Flow-Mediated Vasodilatation in Young Adults Born Very Preterm or With Extremely Low Birthweight: A Regional Cohort Study.

Background: Preterm birth and low birthweight have been associated with increased risk of cardiovascular disease in young adults. Endothelial dysfunction is established as an early marker for development of atherosclerotic cardiovascular disease. Previous studies of endothelial function in young adults born very preterm or with extremely low birthweight have, however, shown diverging results. Objective: We aimed to evaluate the risk of cardiovascular disease as measured by vascular endothelial function in young adults born very preterm (<29 weeks of gestation) or with extremely low birthweight (<1,000 g), compared with term-born controls. Methods: This study included 50 young adults born very preterm or with extremely low birthweight and 49 term-born controls born in Norway in the periods 1982-1985, 1991-1992, and 1999-2000 at mean age 28 (±6) years. The endothelial function was assessed by ultrasound measured flow-mediated dilatation (FMD) of the right brachial artery. The arterial diameter was measured at baseline, after release of 5 min of occlusion, and after sublingual administration of nitroglycerine. FMD was reported as absolute and percentage diameter change from baseline and relative to nitroglycerine-induced dilatation. Results: The participants were mainly normal weight non-smokers, without hypertension, diabetes, or established cardiovascular disease. The cases and controls had mean blood pressure 112/71 (SD 12/9) and 112/69 (SD 11/8) mmHg, body mass index 24.0 (SD 4.2) and 24.4 (SD 4.5) kg/m2, and HbA1c 32.7 (SD 2.5) and 33.0 (SD 2.6) mmol/mol, respectively. For both groups, 4 (8%) were smokers. Mean FMD for the adults born very preterm or with extremely low birthweight was 0.17 mm (95% CI 0.14, 0.21) vs. 0.24 mm (95% CI 0.20, 0.28) for the controls (p = 0.01), corresponding to a percentage increase of 5.4% (95% CI 4.2, 6.6) and 7.6% (95% CI 6.2, 8.9), respectively (p = 0.02). The FMD relative to maximal nitroglycerine-induced dilatation was 20% and 31%, respectively (p = 0.001). Conclusions: Young adults born very preterm or with extremely low birthweight have significantly lower FMD compared with the term-born controls suggesting an increased risk of cardiovascular disease.

Interdisciplinary Approaches Suggested for Children With Multiple Hospital Referrals Presenting With Non-specific Conditions.

Aims: To describe the care patterns of patients with repeated referrals to both mental and somatic specialist healthcare, and to study their diagnostic processes. Methods: In a previous register study patients aged 6-12 years referred to Haukeland University Hospital from 2013 to 2015, we found 922 children with at least three referrals including both somatic and mental health services. Of these, more than one in four (250) were randomly selected and observed from their first hospital episode ever and further after inclusion followed during their next three referrals or until July 2017. Data on referral patterns and diagnostics were collected from patient hospital records. Results: Mean number of referrals was 6.5 prior to inclusion and 4.2 in the follow-up period. At the end of the study period 15% of patients still had a non-specific diagnosis. During the follow-up period, more than half of the children were again referred across the border between somatic and mental healthcare. Conclusion: Very complex care patterns were found for these patients, who were repeatedly being referred and "crossing over" between mental and somatic healthcare. This indicates a need for more interdisciplinary-based approaches both within specialist care and between different care levels to broaden the perspective and achieve shorter time lag before reaching a diagnostic conclusion. Trial Registration: Data was obtained from Haukeland university hospital the patient registry system No. 2017/12470. Start of registration was April 1th 2017 and patients included was from 2013 to 15.

Speckle tracking derived strain in neonates: planes, layers and drift.

The aims of this study was to assess the effect of using a four chamber versus a three plane model on speckle tracking derived global longitudinal strain, the effects of drift compensation, the effect of assessing strain in different layers and finally the interplay between these aspects for the assessment of strain in neonates. Speckle tracking derived longitudinal strain was obtained from 22 healthy neonates. ANOVA, Bland-Altman analyses, coefficients of variation and assessment of intraclass correlation coefficients were conducted to assess the effect of the abovementioned aspects as well as assess both inter-observer and intra-observer variability. Neither the use of the three plane model versus the four chamber model nor the use of drift compensation had a substantial effect on global longitudinal strain (less than 1%, depending on which layer was being assessed). A gradient was seen with increasing strain from the epicardial to endocardial layers, similar to what is seen in older subjects. Finally, drift compensation introduced more discrepancy in segmental strain values compared to global longitudinal strain. Global longitudinal strain in healthy neonates remains reasonably consistent regardless of whether the three plane or four chamber model is used and whether drift compensation is applied. Its value increases when one moves from the endocardial to the epicardial layer. Finally, drift compensation introduces more discrepancy for regional measures of longitudinal strain compared to global longitudinal strain.

Schistosomiasis in Norwegian students after travel to Africa. / Schistosomiasis hos norske studenter etter opphold i Afrika.

BACKGROUND: Schistosomiasis is a tropical infectious disease in which early diagnosis and treatment can prevent serious illness. This study examined the incidence and diagnosis of schistosomiasis in Norwegian exchange students who had been exposed to freshwater in Africa. MATERIAL AND METHOD: Students (n = 318) from Bergen and Oslo who had travelled to Africa as part of an exchange programme in the period 2003-18, were contacted and included in the study if they had been exposed to freshwater during their stay. A routine workup was performed comprising Schistosoma antibody testing, microscopy and/or PCR analysis of urine and faeces, dipstick urinalysis, and blood samples for analysis of eosinophilic granulocytes, creatinine and total IgE. Time, place and type of exposure were recorded in a questionnaire, along with symptoms. RESULTS: Schistosoma antibodies were detected in 46 (30 %) of the 151 students included in the study. None of the seropositive individuals had eggs detected in their urine or faeces, and none had eosinophilia. Two students reported cercarial dermatitis, while one had symptoms consistent with acute schistosomiasis. Rafting was the only form of freshwater exposure reported by 22 (55 %) of the 40 seropositive individuals. INTERPRETATION: A large proportion of the students who had been exposed to freshwater were diagnosed with schistosomiasis. The majority reported no symptoms. Rafting was the most common form of exposure. All were diagnosed by serologic tests, while other routine diagnostic tests for schistosomiasis proved less useful. Serological analysis should be the preferred form of testing for the diagnosis of schistosomiasis in travellers.

Effect of Temporal and Spatial Smoothing on Speckle-Tracking-Derived Strain in Neonates.

Clinical application of strain in neonates requires an understanding of which image acquisition and processing parameters affect strain values. Previous studies have examined frame rate, transmitting frequency, and vendor heterogeneity. However, there is a lack of human studies on how user-regulated spatial and temporal smoothing affect strain values in 36 neonates. This study examined nine different combinations of spatial and temporal smoothing on peak systolic left ventricular longitudinal strain in 36 healthy neonates. Strain values were acquired from four-chamber echocardiographic images in the software-defined epicardial, midwall, and endocardial layers in the six standard segments and average four-chamber stain. Strain values were compared using repeated measure ANOVAs. Overall, spatial smoothing had a larger impact than temporal smoothing, and segmental strain values were more sensitive to smoothing settings than average four-chamber strain. Apicoseptal strain decreased by approximately 4% with increasing spatial smoothing, corresponding to a 13-19% proportional change (depending on wall layer). Therefore, we recommend clinicians be mindful of smoothing settings when assessing segmental strain values.

Bridging the Gap for Children With Compound Health Challenges: An Intervention Protocol.

Background: During the last decades, there is a major shift in the panorama of diseases in children and adolescents. More children are referred to the specialized health care services due to less specific symptoms and more complex health challenges. These children are particularly difficult to care for in a "single-disease" oriented system. Our objective was to develop an alternative and more holistic approach better tailored to the complex needs of these children. Method: The target patient population is children between 6 and 13 years with three or more referrals including both the pediatric department and the mental health services. Furthermore, to be included in the project, the child's actual complaints needed to be clinically considered as an unclear or compound condition in need of an alternative approach. This paper describes the process of developing an intervention where a complementary professional team meets the patient and his/her family altogether for 2.5 h. The consultation focus on clarifying the complex symptomatology and on problem solving. The bio-psycho-social model is applied, emphasizing the patient's story as told on the whiteboard. In the dynamic processes of development, piloting, evaluating, and adjusting the components, feed-back from the patients, their families, professional team members, and external team coaches is important. The professional teams include pediatricians, psychologists and physiotherapists. Achieving the transformation from a logistic oriented team where members act separately toward a real complementary team, seems to be a success factor. Discussion: Composing multi-disciplinary and complementary teams was an essential part of the re-designed intervention. Team interaction transforming the professionals from working as a logistic team to act as a complementary team, was one of the important requirements in the process. When re-designing the specialist health service, it is mandatory to anchor all changes among employees as well as the hospital leadership. In addition, it is important to include patient experiences in the process of improvement. Evaluation of long-term outcomes is needed to investigate possible benefits from the new intervention. Trial Registration: Transitioning Young Patients' Health Care Trajectories, NCT04652154. Registered December 3rd, 2020-Retrospectively registered, https://clinicaltrials.gov/ct2/show/NCT04652154?term=NCT04652154&draw=2&rank=1.

How can we improve specialist health services for children with multi-referrals? Parent reported experience.

BACKGROUND: Children with combined mental and somatic conditions pose a challenge to specialized health services. These cases are often characterized by multi-referrals, frequent use of health services, poor clinical and cost effectiveness, and a lack of coordination and consistency in the care. Reorganizing the health services offered to these children seems warranted. Patient reported experiences give important evidence for evaluating and developing health services. The aim of the present descriptive study was to explore how to improve specialist health services for children with multiple referrals for somatic and mental health conditions. Based on parent reported experiences of health services, we attempted to identify key areas of improvement. METHODS: As part of a larger, ongoing project; "Transitioning patients' Trajectories", we asked parents of children with multiple referrals to both somatic and mental health departments to provide their experiences with the services their children received. Parents/guardians of 250 children aged 6-12 years with multi-referrals to the Departments of Pediatrics and Child and Adolescent Mental Health at Haukeland University Hospital between 2013 and 2015 were invited. Their experience was collected through a 14 items questionnaire based on a generic questionnaire supplied with questions from parents and health personnel. Possible associations between overall experience and possible predictors were analyzed using bivariate regression. RESULTS: Of the 250 parents invited, 148 (59%) responded. Mean scores on single items ranged from 3.18 to 4.42 on a 1-5 scale, where five is the best possible experience. In the multiple regression model, perception of wait time (r = .56, CI = .44-.69 / ß = 0.16, CI = .05-.28), accommodation of consultations (r = .71, CI = .62-.80 / ß = 0.25, CI = .06-.45 / ß = 0.27, CI = .09-.44), providing adequate information about the following treatment (r = .66, CI = .55-.77 / ß = 0.26, CI = .09-.43), and collaboration between different departments at the hospital (r = .68, CI = .57-.78 / ß = 0.20, CI = -.01-.40) were all statistically significantly associated with parents overall experience of care. CONCLUSIONS: The study support tailored interdisciplinary innovations targeting wait time, accommodation of consultations, communication regarding the following treatment and collaboration within specialist health services for children with multi-referrals to somatic and mental specialist health care services.

Complex care pathways for children with multiple referrals demonstrated in a retrospective population-based study.

AIM: To identify children with complex medical needs by examining their patterns of hospital care. METHODS: We conducted a retrospective population-based study on 18 577 patients aged 6-12 years from the Haukeland University Hospital register over a 3-year period (from 2013 to 2015). Data were structured to examine the temporal patterns and sequences of referrals, care episodes and diagnoses, including flow across medical specialties. RESULTS: Over a third of patients had repeated referrals, and 14.9% of all had three or more. Furthermore, 9.3% of patients were referred to both somatic and mental healthcare services. Patients with such combined referrals had a higher number of referrals as well as a higher number of different diagnoses. Overall, there was a high frequency of non-specific diagnoses, and 34.8% of patients still had a non-specific main diagnosis at the end of their hospital contact. CONCLUSION: This study demonstrates an increased risk for complex care pathways in children with multiple referrals. Interdisciplinary patterns of referrals were relatively common, particularly for patients in mental health care. These findings highlight the importance of developing interdisciplinary-based approaches for patients with complex complaints.

What is Left Ventricular Strain in Healthy Neonates? A Systematic Review and Meta-analysis.

Reference values for left ventricular systolic strain in healthy neonates are necessary for clinical application of strain. The objectives of this systematic review were to identify echocardiographic studies that presented left ventricular strain values in healthy neonates, perform a meta-analysis for speckle tracking-derived global longitudinal strain, and identify areas that require further investigation. A structured search was applied to MEDLINE, Embase, and Cochrane Central Register of Clinical Trials in search of echocardiographic studies that presented left ventricular strain in healthy neonates. 244 studies were identified, of which 16 studies including speckle tracking and tissue Doppler strain in the longitudinal, radial, and circumferential directions passed the screening process. Out of these 16 studies, a meta-analysis was performed on the 10 studies that reported speckle tracking global longitudinal strain. Mean speckle tracking-derived global longitudinal strain was 21.0% (95% Confidence Interval 19.6-22.5%, strain given as positive values). When the studies were divided into subgroups, mean speckle tracking global longitudinal strain from the four-chamber view was 19.5% (95% Confidence Interval 18.0-21.0%) and that derived from all three apical views was 22.5% (95% CI 20.6-24.7%), indicating that global longitudinal strain from the four-chamber view is slightly lower than global longitudinal strain from all three apical views. Neonatal strain values were close to strain values in older subjects found in previous meta-analyses. Further studies are recommended that examine strain rate, segmental strain values, strain derived from short axis views, and strain in the first few hours after birth.

Cardiopulmonary Capacity in Children During Exercise Testing: The Differences Between Treadmill and Upright and Supine Cycle Ergometry.

Background/Hypothesis: Cardiopulmonary exercise testing (CPET) is used in the assessment of function and prognosis of cardiopulmonary health in children with cardiac and pulmonary diseases. Techniques, such as cardiac MRi, and PET-scan, can be performed simultaneously with exercise testing. Thus, it is desirable to have a broader knowledge about children's normal cardiopulmonary function in different body postures and exercise modalities. The aim of this study was to investigate the effect of different body positions on cardiopulmonary function in healthy subjects performing CPETs. Materials and Methods: Thirty-one healthy children aged 9, 12, and 15 years did four CPETs: one treadmill test with a modified Bruce protocol and three different bicycle tests with different body postures, sitting, tilted 45°, and lying flat (0°). For the bicycle tests, a 20-watt ramp protocol with a pedal frequency of 60 ± 5 rotations per minute was used. Continous ECG and breath-by-breath V . O 2 measurements was done throughout the tests. Cardiac structure and function including aortic diameter were evaluated by transthoracic echocardiography prior to the tests. Doppler measurements of the blood velocity in the ascending aorta were measured prior to and during the test. Prior to every test, the participants performed pulmonary function tests with maximum voluntary ventilation test. Results: There is a significantly (p < 0.05) lower peak V . O 2 in all bicycle tests compared with the treadmill test. There is lower corrected peak V . O 2 (ml kg-0.67 min-1), but not relative peak V . O 2 (ml kg-1 min-1), in the supine compared with the upright bicycle test. There are no differences in peak stroke volume or cardiac output between the bicycle modalities when calculated from aortic blood flow. Peak heart rate decreases from both treadmill to upright bicycle and from upright bicycle to the supine test (0°). Conclusion: There are no differences in peak cardiac output between the upright bicycle test and supine bicycle tests. Heart rate and corrected peak V . O 2 are lower in the supine test (0°) than the upright bicycle test. In the treadmill test, it is a higher absolute and relative peak V . O 2 . Despite the latter differences, we are convinced that both upright and supine bicycle tests are apt in the clinical setting when needed.

Effectiveness of an oral health intervention program for children with congenital heart defects.

BACKGROUND: Children with congenital heart defects (CHD) are reported to have poorer oral health compared with healthy children. The aim of the present study was to evaluate the effectiveness of an intensive oral health care program among children with CHD followed from infancy to the age of 5 years, by comparing their oral health status at 5 years with a control group of children with CHD who had not received the program. METHODS: In this longitudinal study, children in western Norway with a need for lifelong follow-up due to congenital heart defects were invited to participate (n = 119). Children born in 2008-2011 were offered an oral health intervention program from infancy to the age of 5 years. The outcome measures for evaluating the intervention were dental caries prevalence, dental erosion, plaque index and gingival bleeding index. The data of the intervention group were compared with cross sectional oral health data of 5 year old controls with CHD born 2005-2007 (already published). RESULTS: Early oral health intervention did not affect the prevalence of caries (25.3% versus 25.4%) or dental erosion (22.2% versus 19.7%) of children with CHD assessed at 5 years. Children in the intervention group were less likely than those in the control group to present with both dental plaque and gingival bleeding at age 5 years. In spite of no difference in caries prevalence between the groups, caries affected children (d1-5mft) in the intervention group had fewer teeth affected by caries than children in the control group (p = 0.06). The care index was reported to be higher in the intervention group compared with the control group, implying that fewer children in the intervention group suffered from untreated dentine caries. Parents in the intervention group were more likely to brush their children's teeth twice a day than parents of children in the control group. CONCLUSION: The oral health promotive program did not influence the prevalence of caries nor dental erosion. However, the findings indicated better oral hygiene, reduced gingival bleeding and less untreated dentine caries in the intervention compared with the control group. TRIAL REGISTRATION: ClinicalTrials.gov NCT03311438 . Registration date: October 17th 2017, retrospectively registered.

The five-year survival of children with Down syndrome in Norway 1994-2009 differed by associated congenital heart defects and extracardiac malformations.

AIM: We investigated the prevalence of Down syndrome in a nationwide birth cohort, focusing on congenital heart defects (CHDs), their associations with extracardiac malformations (ECM) and survival. METHODS: National registers were used to identify Norwegian births (1994-2009) and deaths (1994-2014) and updated with hospital diagnoses. We estimated birth defect frequencies in Down syndrome and the general population, the association between CHDs and ECM and hazard ratios for death from different combinations of CHDs and ECM. RESULTS: Down syndrome was found in 1672 of 953 450 births (17.6 per 10 000). Of the 1251 live births (13.3 per 10 000), 58% had CHD and 9% ECM. CHDs were associated with oesophageal atresia (p = 0.02) and Hirschsprung's disease (p = 0.03) but with no other malformations. The five-year survival for Down syndrome increased from 91.8% (1994-1999) to 95.8% (2000-2009) (p = 0.006), and overall survival was 92.0% with CHD and 97.4% without. Compared with Down syndrome children without CHD or ECM, the five-year mortality was similar for those with nonsevere CHDs, without or with ECM, but 4-7 times higher in those with severe CHDs without ECM and 13-28 times higher in those with severe CHDs and ECM. CONCLUSION: Down syndrome childhood survival improved, but mortality remained high with severe CHDs and extracardiac defects.

Recurrence of congenital heart defects among siblings-a nationwide study.

Congenital heart defects (CHD) constitute the largest group of congenital malformations. In most families, only one person has CHD; however, the risk of CHD increases for children born into families already affected. In this study, all births from 1994 through 2009 were identified in the Medical Birth Registry of Norway, including supplemental information on CHD from clinical and administrative registers, as part of the CVDNOR project. By using the unique personal identification number of each parent we were able to link 16,078 pairs of twins, 445,584 pairs of full siblings, and 106,840 pairs of half-siblings. Sibling recurrence risk ratio (RRR) was calculated using CHD status in the oldest sibling as exposure and CHD status in the younger sibling as outcome, adjusted for year of birth, maternal age, and maternal diabetes. Among full sibling pairs with CHD in the older sibling, the younger sibling had CHD in 4.1% compared to 1.1% of the pairs without CHD in the older sibling (adjusted RRR 3.6; 95% confidence interval (CI) 3.1-4.1). In same-sex twins the RRR was 14.0 (95% CI 10.6-18.6), and in opposite-sex twins the RRR was 11.9 (95% CI 7.1-19.9). For half-siblings the RRR was 1.5 (95% CI 0.8-2.8). When restricting to severe types of CHD, the RRR was 6.9 (95% CI 4.9-9.8) for full siblings. In 50% of the pairs with recurrent CHD, the siblings had similar types of CHD. The high relative risk of recurrence indicates that familial risk factors are important in the etiology of CHD.