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BACKGROUND Approximately 1% of all infants are born with a congenital heart disease (CHD). Internationally CHD remains a major cause of infant death, some of which occur unexpectedly after a gradual deterioration at home. Many parents find it difficult to recognize worsening of symptoms. OBJECTIVE This study aims to report the acceptability and initial adoption of a mobile app, the Heart Observation app (HOBS), aiming to support parents’ understanding and management of their child’s condition and to increase quality in follow-up from health care professionals in complex health care services in Norway. METHODS A total of 9 families were interviewed on discharge from the neonatal intensive care unit and after 1 month at home. The infant’s primary nurse, community nurse, and cardiologist were also interviewed regarding their experiences about collaboration with the family. The interviews were analyzed inductively with thematic content analysis. RESULTS The analysis generated 4 main themes related to acceptability and adoption: (1) Individualize Initial Support, (2) Developing Confidence and Coping, (3) Normalize When Appropriate, and (4) Implementation in a Complex Service Pathway. The receptivity of parents to learn and attend in the intervention differs according to their present situation. Health care professionals emphasized the importance of adapting the introduction and guidance to parents’ receptivity to ensure comprehension, self-efficacy, and thereby acceptance before discharge (Individualize Initial Support). Parents perceived that HOBS served them well and nurtured confidence by teaching them what to be aware of. Health care professionals reported most parents as confident and informed. This potential effect increased the possibility of adoption (Developing Confidence and Coping). Parents expressed that HOBS was not an “everyday app” and wanted to normalize everyday life when appropriate. Health care professionals suggested differentiating use according to severity and reducing assessments after recovery to adapt the burden of assessments when appropriate (Normalize When Appropriate). Health care professionals’ attitude to implement HOBS in their services was positive. They perceived HOBS as useful to systemize guidance, to enhance communication regarding an infant’s condition, and to increase understanding of heart defects in health care professionals with sparse experience (Implementation in a Complex Service Pathway). CONCLUSIONS This feasibility study shows that both parents and health care professionals found HOBS as a positive addition to the health care system and follow-up. HOBS was accepted and potentially useful, but health care professionals should guide parents initially to ensure comprehension and adapt timing to parents’ receptivity. By doing so, parents may be confident to know what to look for regarding their child’s health and cope at home. Differentiating between various diagnoses and severity is important to support normalization when appropriate. Further controlled studies are needed to assess adoption, usefulness, and benefits in the health care system.

AimsUpdated knowledge on the rates and causes of death among children with severe congenital heart defects (CHDs) is needed to further improve treatment and survival. This study investigated nationwide mortality rates in children with severe CHDs with an emphasis on unexpected mortality unrelated to cardiac intervention.Methods and resultsData on all pregnancies and live-born children in Norway from 2004 to 2016 were obtained from national registries, the Oslo University Hospital’s Clinical Registry for CHDs and medical records. Among 2359 live-born children with severe CHDs, 234 (10%) died before 2 years of age. Of these, 109 (46%) died in palliative care, 58 (25%) died of causes related to a cardiac intervention and 67 (29%) died unexpectedly and unrelated to a cardiac intervention, either before (n=26) or following (n=41) discharge after a cardiac intervention. Comorbidity (38/67, 57%), persistent low oxygen saturation (SaO2; ConclusionsThe numbers of unexpected deaths unrelated to cardiac intervention in children

OBJECTIVES This population-based, comprehensive, retrospective study presented the clinical outcomes of all children born in Norway between 2003 and 2017 with double outlet right ventricle (DORV). METHODS All children born with DORV between 2003 and 2017 were identified in the Oslo University Hospital registry. Patients’ characteristics, interventions, complications and deaths were recorded. Echocardiographic data were reviewed for classification according to current standards. We investigated time-dependent surgical reintervention and mortality using Kaplan–Meier analyses and determinants of treatment complications, reintervention and death using regression analyses. RESULTS Ninety-three children with DORV represented an annual median prevalence of 1.18 per 10 000 births in Norway. Six children received palliative care. With an intention to treat, a surgical route with the primary biventricular repair was followed for 62 children, staged biventricular repair for 15 and univentricular repair for 10 children. Major complications occurred in 1.0% and 6.2% of children following catheter or surgical intervention, respectively. No significant determinants of the complications were identified. Overall survival following treatment was 91.9%, 90.8%, 89.5% and 89.5% and corresponding freedom from surgical reintervention was 88.0%, 79.0%, 74.9% and 69.4% at 1, 2, 5 and 10 years, respectively. The presence of atrioventricular septal defect predicted an increased risk of mortality (hazard ratio: 7.16) but did not increase the risk of surgical reintervention. CONCLUSIONS In Norway, most children receive tailored treatment for DORV with low rates of complications, surgical reinterventions and mortality. However, atrioventricular septal defect remains a potential determinant of postoperative death.

Background and objectives: Many parents of infants with CHD find it difficult to recognise symptoms of deterioration in their children. Therefore, a personalised decision support application for parents has been developed. This application aims to increase parents’ awareness of their infant’s normal condition, help them assess signs of deterioration, decide who and when to contact health services, and what to report. The aim of this paper is to describe the concept and report results from a usability study. Methods: An interprofessional group developed a mobile application called the Heart OBServation app in close collaboration with parents using an iterative process. We performed a usability study consisting of semi-structured interviews of 10 families at discharge and after one month and arranged two focus group interviews with nurses caring for these families. A thematic framework analysis of the interviews explored the usability of features in the application. Usability was assessed twice using the System Usability Scale, and a user log was registered throughout the study. Results: The overall system usability score, 82.3 after discharge and 81.7 after one month, indicates good system usability. The features of Heart OBServation were perceived as useful to provide tailored information, increase awareness of the child’s normal condition, and to guide parents in what to look for. To empower parents, an interactive discharge checklist was added. Conclusions: The Heart OBServation demonstrated good usability and was well received by parents and nurses. Feasibility and benefits of this application in clinical practice will be investigated in further studies

Background and aimsSevere congenital heart defects (CHDs) still represent one of the main causes of infant death. The risk factors associated with cardiac surgery and postoperative mortality are well known. We aimed to describe the rates, causes and time trends of mortality before surgery—including termination of pregnancies and palliative care—in fetuses and children below 2 years of age with severe CHDs.Methods and resultsData concerning all 789 345 pregnancies in Norway from 2004 to 2016 were retrieved from the Medical Birth Registry of Norway, the Oslo University Hospital’s Clinical Registry for Congenital Heart Defects, the Norwegian Cause of Death Registry, the National Registry, Statistics Norway, autopsy reports and medical records. When including termination of pregnancy and stillbirths, the number of fetuses and children with severe CHDs that did not reach the age of 2 years was 31%. Mortality among the 2359 live-born infants with severe CHDs was 10%, of whom 58% died before surgery. Of the preoperative deaths, 81% died in a palliative care setting, and comorbidity and univentricular CHDs were common among these infants. Together, palliative care and termination of pregnancy accounted for 86% of deaths in cases of severe CHDs, and this proportion increased during the study period (annual percent changes 1.3, 95% CI 0.4 to 2.1, pConclusionsTermination of pregnancy accounted for the majority of the deaths in fetuses and children with severe CHDs. Among live-born children, most preoperative deaths occurred in a palliative care setting and were strongly related to comorbidities and/or univentricular hearts.

The role of dysfunction of the single ventricle in Fontan failure is incompletely understood. We aimed to evaluate hemodynamic responses to preload increase in Fontan circulation, to determine whether circulatory limitations in different locations identified by experimental preload increase are associated with cardiorespiratory fitness (CRF), and to assess the impact of left versus right ventricular morphology. In 38 consecutive patients (median age = 16.6 years, 16 females), heart catheterization was supplemented with a rapid 5-mL/kg body weight volume expansion. Central venous pressure (CVP), ventricular end-diastolic pressure (VEDP), and peak systolic pressure were averaged for 15‒30 s, 45‒120 s, and 4‒6 min (steady state), respectively. CRF was assessed by peak oxygen consumption (VO2peak) and ventilatory threshold (VT). Median CVP increased from 13 mmHg at baseline to 14.5 mmHg (p p 2peak and VT. VEDP after volume expansion was associated with VT. Preload challenge demonstrates the limitations beyond baseline measurements. Elevation of both CVP and VEDP are associated with impaired CRF. Transpulmonary flow limitation was more pronounced in right ventricular morphology. Ventricular dysfunction may contribute to functional impairment after Fontan operation in young adulthood.ClinicalTrials.govidentifier NCT02378857

Purpose Patients with Fontan circulation are at risk of developing hepatic fibrosis/cirrhosis. The mechanisms and disease development are unclear and early secondary liver cancer is a concern. This study will describe hepatic imaging findings in a national cohort of adolescents with Fontan circulation. Methods The patients prospectively underwent abdominal contrast enhanced magnetic resonance imaging (MRI) including diffusion-weighted imaging. Images were assessed for criteria of fibrosis/cirrhosis including characterization of hepatic nodules. These nodules were in addition, assessed by ultrasonography (US). Nodules ≥ 1 cm were investigated and monitored to evaluate malignant transformation. Clinical and hepatic serological data were recorded. Results Forty-six patients, median age of 16.5 years (15.4–17.9 years) were enrolled. All patients underwent US examination and MRI was performed in 35/46 patients. On MRI, 60% had hepatomegaly and 37% had signs of fibrosis/cirrhosis. Seven patients had together 13 nodules ≥ 1 cm in diameter. Only 4/13 (17%) where seen on US. Nodules had variable MRI signal characteristics including hepatobiliary contrast enhancement and two nodules revealed portal venous phase ‘wash-out’ on the first examination. No further imaging signs of malignancy were revealed during the follow-up period of median 24.4 (7–42) months. Conclusion The majority of adolescents with Fontan circulation had imaging findings of fibrosis/cirrhosis of varying severity. US had low detection rate of hepatic nodules compared to MRI. The imaging work-up before transition to adult cardiology care did not reveal findings suggestive of malignancy. However, the high prevalence of Fontan-associated liver disease calls for surveillance strategies even in childhood.

This paper presents an experimental investigation of injection-moulded short glass-fibre reinforced polyamide-6 reinforced with 0 wt.%, 15 wt.% and 30 wt.% fibres. The fibre orientation distributions are characterized by use of X-ray computed tomography. A shell-core-shell structure is found through the thickness of the materials, where the predominant fibre orientation is along the mould flow direction (MFD) in the shell layers and perpendicular to the MFD in the core layer. To study the mechanical behaviour, uniaxial tensile tests are conducted in seven directions relative to the MFD. The tests are instrumented with two cameras, which allows for accurate measurement of all strain components. The fibre-reinforced materials show moderate to high degree of anisotropy, increasing with the fibre content. Young’s modulus, in-plane and out-of-plane Poisson’s ratio, stress at maximum force and fracture strain vary smoothly with the in-plane specimen angle. It is demonstrated that orthotropic elasticity is an excellent approximation for the anisotropic elastic behaviour of the fibre-reinforced materials. © 2020 The Author(s). Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license

This article presents a simple progressive damage model for quasi-brittle materials, combining orthotropic elasticity with a scalar damage model including spatial variation of the damage initiation strain and the crack band method for softening regularization. The model’s performance is first analyzed from a numerical point of view and then demonstrated for tensile tests ( 0 ° , 45 ° and 90 ° ), open-hole tensile tests ( 0 ° ) and three-point bending ( 0 ° and 90 ° ) tests of short fibre-reinforced polypropylene with 15 wt.% and 30 wt.% glass fibres. Despite its simplicity, the model captures the anisotropic elastic and inelastic behaviour observed in experiments. The model is applicable for orthotropic brittle or quasi-brittle materials, where the variability in elastic properties is negligible and the orientation dependency of the fracture strain is small or not relevant for the application.

OBJECTIVES Following paediatric cardiac surgery with cardiopulmonary bypass (CPB), there is a tendency for fluid accumulation. The colloid osmotic pressure of plasma (COPp) and interstitial fluid (COPi) are determinants of transcapillary fluid exchange but only COPp has been measured in sick children. The aim of this study was to assess the net colloid osmotic pressure gradient in children undergoing atrial septal defect closure. METHODS Twenty-three patients had interventional and 18 had surgical atrial septal defect closures. Interstitial fluid was harvested using a wick method before and after surgery with CPB with concomitant blood samples. COP was measured using a colloid osmometer for small fluid samples. Baseline COP was compared with data from healthy children. RESULTS COPp at baseline was 21.9 ± 2.8 and 21.4 ± 2.2 mmHg in the interventional and surgical groups, respectively, and was significantly lower than in healthy children (25.5 ± 3.1 mmHg) (P

AimsOut-of-hospital sudden cardiac arrest (SCA) is a rare but devastating event in children and adolescents. The risk is assumed to be higher in children with congenital heart defects (CHDs) than in healthy individuals. The aim of the present study was to investigate the rate of and survival after out-of-hospital cardiac arrest in children 2–18 years old with CHDs.Methods and resultsData concerning all live births in Norway between 1994 and 2009 were retrieved from the Medical Birth Registry of Norway, the patient administrative systems at all hospitals in Norway, the Oslo University Hospital’s Clinical Registry for Congenital Heart Defects and the Norwegian Cause of Death Registry. Survivors were followed through 2012, and supplementary information for the deceased children was retrieved from medical records at Norwegian hospitals. Among the 943 871 live births in Norway from 1994 to 2009, 11 272 (1.2%) children had a CHD. We identified 11 (0.1%) children 2–18 years old with CHDs who experienced out-of-hospital SCA. The estimated rate of out-of-hospital SCA in children 2–18 years old with CHD was 10 per 100 000 person-years. Early cardiopulmonary resuscitation was initiated in all patients. Three children survived.ConclusionsThe incidence of and survival after out-of-hospital SCA in children with CHDs were comparable to the reported rates in the general child population.

Most structural congenital heart defects can be identified prenatally through ultrasound examination in pregnancy or via routine examinations during hospital maternity stays, but in some cases, heart defects are not discovered prior to discharge. There has been little previous research into detection rates with the various methods available. In this study, we have examined the timing and method of diagnosis of severe congenital heart defects.All children with severe heart defects born in Norway in 2016 and registered at Oslo University Hospital were included in this study. In addition, information on committee-handled abortions (after the 12th week of pregnancy) was obtained from the Medical Birth Registry of Norway.In total, 105 of 181 (58 %) severe heart defects were diagnosed prenatally, and 51 (28 %) pregnancies were terminated. Among the 73 live-born children with severe heart defects that went unrecognised prenatally, 33 (45 %) of the heart defects were discovered outside of routine examinations and 9 (12 %) after discharge from hospital. Coarctation of the aorta was the most common diagnosis in cases of late-detected heart defects.This first national study of the diagnosis of severe congenital heart defects in Norway shows that most severe congenital heart defects are discovered prior to discharge from hospital after birth. However, almost half are diagnosed outside of routine examinations, and in some cases the diagnosis is not made until after discharge. The results indicate a need for new studies and for a quality registry of congenital heart defects to further improve diagnosis and early treatment.

Studies report increased risk of congenital heart defects (CHD) in the offspring of mothers with diabetes, where high blood glucose levels might confer the risk. We explored the association between intake of sucrose-sweetened soft beverages during pregnancy and risk of CHD. Prospective cohort data with 88,514 pregnant women participating in the Norwegian Mother and Child Cohort Study was linked with information on infant CHD diagnoses from national health registers and the Cardiovascular Diseases in Norway Project. Risk ratios were estimated by fitting generalized linear models and generalized additive models. The prevalence of children with CHD was 12/1000 in this cohort (1049/88,514). Among these, 201 had severe and 848 had non-severe CHD (patent ductus arteriosus; valvular pulmonary stenosis; ventricular septal defect; atrial septal defect). Only non-severe CHD was associated with sucrose-sweetened soft beverages. The adjusted risk ratios (aRR) for non-severe CHD was 1.30 (95% CI 1.07–1.58) for women who consumed 25–70 ml/day and 1.27 (95% CI 1.06–1.52) for women who consumed ≥ 70 ml/day when compared to those drinking ≤ 25 ml/day. Dose–response analyses revealed an association between the risk of non-severe CHD and the increasing exposure to sucrose-sweetened soft beverages, especially for septal defects with aRR = 1.26 (95% CI 1.07–1.47) per tenfold increase in daily intake dose. The findings persisted after adjustment for maternal diabetes or after excluding mothers with diabetes (n = 19). Fruit juices, cordial beverages and artificial sweeteners showed no associations with CHD. The findings suggest that sucrose-sweetened soft beverages may affect the CHD risk in offspring. acceptedVersion

Background Most structural congenital heart defects can be identified prenatally through ultrasound examination in pregnancy or via routine examinations during hospital maternity stays, but in some cases, heart defects are not discovered prior to discharge. There has been little previous research into detection rates with the various methods available. In this study, we have examined the timing and method of diagnosis of severe congenital heart defects. Material and method All children with severe heart defects born in Norway in 2016 and registered at Oslo University Hospital were included in this study. In addition, information on committee-handled abortions (after the 12th week of pregnancy) was obtained from the Medical Birth Registry of Norway. Results In total, 105 of 181 (58 %) severe heart defects were diagnosed prenatally, and 51 (28 %) pregnancies were terminated. Among the 73 live-born children with severe heart defects that went unrecognised prenatally, 33 (45 %) of the heart defects were discovered outside of routine examinations and 9 (12 %) after discharge from hospital. Coarctation of the aorta was the most common diagnosis in cases of late-detected heart defects. Interpretation This first national study of the diagnosis of severe congenital heart defects in Norway shows that most severe congenital heart defects are discovered prior to discharge from hospital after birth. However, almost half are diagnosed outside of routine examinations, and in some cases the diagnosis is not made until after discharge. The results indicate a need for new studies and for a quality registry of congenital heart defects to further improve diagnosis and early treatment.

The aim of this prospective study was to determine the frequency and composition of cerebral microemboli in a pediatric population, during transcatheter closure of patent ductus arteriosus (PDA). Multifrequency transcranial Doppler was used to monitor cerebral blood flow velocity (CBFV) and detect microembolic signals (MES) in the middle cerebral artery in 23 patients (median age 18 months). MES were automatically identified and differentiated according to composition; gaseous or solid. The procedure was divided into five periods: Arterial catheterization; venous catheterization; ductal catheterization; angiography; device placement and release. Timing of catheter manipulations and MES were registered and compared. MES were detected in all patients. The median number of signals was 7, (minimum 1, maximum 28). Over 95 % of all MES were gaseous. 11 % were detected during device placement while 64 % were detected during angiographic studies, significantly higher than during any other period (P

Objective The aim of the present nationwide cohort study was to describe trends in 1-year mortality in live-born children with congenital heart defects in Norway 1994-2009 and to assess whether changes in the proportion of terminated pregnancies and altered operative mortality have influenced these trends. Methods Medical information concerning all 954 413 live births, stillbirths, and late-term abortions in Norway, 1994-2009, was retrieved from the Medical Birth Registry of Norway, the Cardiovascular Disease in Norway project, the Oslo University Hospital's Clinical Registry for Congenital Heart Defects and the Norwegian Cause of Death Registry. Survivors were followed through 2012. Results The 1-year cumulative mortality proportion during the study period was 17.4% for children with severe congenital heart defects and 3.0% for children with nonsevere congenital heart defects. The 1-year cumulative mortality proportion among live born children with severe congenital heart defects decreased 3.6% (95% CI: -5.4, -1.5) per year. The total mortality of severe congenital heart defects was unchanged when including stillbirths and late-term abortions with severe congenital heart defects. The proportion of stillbirths or terminated pregnancies with severe congenital heart defects among all pregnancies with severe congenital heart defects, was on average 8.8% over the entire period with an annually increase of 16.6% (11.4, 18.0). The mean operative mortality in children with severe congenital heart defects was 8.4% and decreased by 9.0% (-11.9, -5.9) per year. Conclusions The 1-year mortality of severe congenital heart defects among live births, 1994-2009, declined in Norway. The downward trend in mortality may be explained by a more frequent use of termination of affected pregnancies, and the reduced operative mortality of severe congenital heart defects.

Background The aetiology of congenital heart defects (CHD) is mostly unknown, but maternal factors may modify the infant risk of CHD. We investigated the association between maternal preeclampsia and offspring risk of severe CHD in a nation-wide cohort study. Methods Information on all births registered in the Medical Birth Registry of Norway, 1994–2009, was completed with information on CHD diagnoses from national health registries and the Cardiovascular Diseases in Norway Project (CVDNOR). Results Among 914 703 singleton births without chromosomal abnormalities, 32 864 (3.6%) were born after a pregnancy with preeclampsia. The preeclampsia was diagnosed before the 34th week of pregnancy (early-onset preeclampsia) in 2618 (8.0% of preeclamptic pregnancies). CHDs were diagnosed in 10 691 infants; of these, 2473 had severe CHD. The risk of severe CHD was compared between births with and without maternal preeclampsia and estimated with binomial log-linear regression. When adjusting for year of birth, maternal age, parity, and pregestational diabetes, the risk ratio (RR) for severe CHD in offspring of mothers with any preeclampsia was 1.3 [95% confidence interval (CI) 1.1, 1.5], and in pregnancies with early-onset preeclampsia, the RR was 2.8 (95% CI 1.8, 4.4). The association between early-onset preeclampsia and specific types of severe CHD was stronger for atrioventricular septal defects (AVSD), with adjusted RR 13.5 (95% CI 6.8, 26.8). Conclusions Early-onset preeclampsia was strongly associated with infant risk of severe CHD, specifically; the risk of AVSD was 15-fold higher if the mother was diagnosed with early-onset preeclampsia, suggesting common aetiological factors for early-onset preeclampsia and erroneous fetal heart development.

OBJECTIVES Despite Fontan surgery showing improved results, fluid accumulation and oedema formation with pleural effusion are major challenges. Transcapillary fluid balance is dependent on hydrostatic and colloid osmotic pressure (COP) gradients; however, the COP values are not known for Fontan patients. The aim of this study was to evaluate the COP of plasma (COPp) and interstitial fluid (COPi) in children undergoing bidirectional cavopulmonary connection and total cavopulmonary connection. METHODS This study was designed as a prospective, observational study. Thirty-nine children (age 3 months-4.9 years) undergoing either bidirectional cavopulmonary connection or total cavopulmonary connection procedures were included. Blood samples and interstitial fluid were obtained prior to, during and after the preoperative cardiac catheterization and surgery with the use of cardiopulmonary bypass (CPB). Interstitial fluid was harvested using the wick method when the patient was under general anaesthesia. Plasma and interstitial fluid were measured by a colloid osmometer. Baseline values were compared with data from healthy controls. RESULTS Baseline COPp was 20.6 ± 2.8 and 22.0 ± 3.2 mmHg and COPi was 11.3 ± 2.6 and 12.5 ± 3.5 mmHg in the bidirectional cavopulmonary connection group and the total cavopulmonary connection group, respectively. These values were significantly lower than in healthy controls. The COPp was slightly reduced throughout both procedures and normalized after surgery. The COPi increased slightly during the use of CPB and significantly decreased after surgery, resulting in an increased COP gradient and was correlated to pleural effusion. CONCLUSIONS Fluid accumulation seen after Fontan surgery is associated with changes in COPs, determinants for fluid filtration and lymphatic flow. CLINICALTRIALS.GOV IDENTIFIER NCT 02306057: https://clinicaltrials.gov/ct2/results?cond=&term=NCT+02306057.

Objectives Congenital heart defects (CHD) are the most common birth defects worldwide and are an important cause of morbidity and early death. A significant number of deaths occur among patients with infections. CHDs predispose to the development of infective endocarditis (IE) and represent a risk factor for increased mortality due to IE. The aim of this study was to investigate the occurrence and outcomes of IE in children and adolescents with CHDs. Methods Data on all children with CHD and IE born in Norway between 1994 and 2016 were retrieved from the Oslo University Hospital’s Clinical Registry for Congenital Heart Defects. Survivors were followed through 2016, and supplementary information was retrieved from medical records. Results In this nationwide register-based cohort study, which included all 1 357 543 live births in Norway between 1994 and 2016, the incidence of IE according to the European Society of Cardiology diagnostic criteria was 2.2 per 10 000 person-years among children and adolescents with CHDs. The incidence was stable throughout the period. Most patients with IE had severe CHDs (75%) and had undergone open chest cardiac surgery or catheter-based cardiac interventions the last year before IE. IE-related mortality among children with CHDs and IE was 8% during the follow-up period (mean 12.4 years (±5.5 years)). Conclusions The incidence of IE among children and adolescents with CHDs was higher than the reported incidence in the general population. IE was associated with severe CHDs and recent complex cardiac interventions, and had significant mortality.

We previously demonstrated an abnormally high right ventricular systolic pressure response to exercise in 50% of adolescents operated on for isolated ventricular septal defect. The present study investigated the prevalence of abnormal right ventricular systolic pressure response in 20 adult (age 30–45 years) patients who underwent surgery for early ventricular septal defect closure and its association with impaired ventricular function, pulmonary function, or exercise capacity. The patients underwent cardiopulmonary tests, including exercise stress echocardiography. Five of 19 patients (26%) presented an abnormal right ventricular systolic pressure response to exercise ⩾ 52 mmHg. Right ventricular systolic function was mixed, with normal tricuspid annular plane systolic excursion and fractional area change, but abnormal tricuspid annular systolic motion velocity (median 6.7 cm/second) and isovolumetric acceleration (median 0.8 m/second2). Left ventricular systolic and diastolic function was normal at rest as measured by the peak systolic velocity of the lateral wall and isovolumic acceleration, early diastolic velocity, and ratio of early diastolic flow to tissue velocity, except for ejection fraction (median 53%). The myocardial performance index was abnormal for both the left and right ventricle. Peak oxygen uptake was normal (mean z score −0.4, 95% CI −2.8–0.3). There was no association between an abnormal right ventricular systolic pressure response during exercise and right or left ventricular function, pulmonary function, or exercise capacity. Abnormal right ventricular pressure response is not more frequent in adult patients compared with adolescents. This does not support the theory of progressive pulmonary vascular disease following closure of left-to-right shunts.

Aim This study determined the use of standardised procedures for infant noninvasive blood pressure (NIBP) measurements in the Nordic countries and aimed to identify factors included in the standardisation and interpretation of NIBP measurements in infants. Methods A cross-sectional electronic questionnaire survey was sent to 84 physicians in all 23 university hospitals in Sweden, Norway, Denmark, Finland and Iceland and was completed from February to March 2017. The survey contained respondent characteristics, the presence and description of standardised procedures for NIBP measurements, daily practice of NIBP measurements and methodological considerations and interpretation of NIBP measurements in a healthy six-month-old child. Results We received responses from 55 of 84 physicians working in all 23 Nordic university hospitals, in paediatric cardiology (n = 22), general paediatrics (n = 16), paediatric nephrology (n = 14) and other fields (n = 3). Less than a quarter (23%) said their hospital issued specific NIBP procedures relating to infants and they referred to 19 different sources of information. The factors that were most commonly assessed for interpretation were age (100%), arousal state (78%) and cuff size (76%). Conclusion Most of the university hospital units treating children lacked age-specific written procedures for measuring and interpreting infant NIBP, and there is a strong need for common Nordic guidelines.

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.

Background: The reasons for decreasing birth prevalence of congenital heart defects (CHDs) in several European countries and Canada are not fully understood. We present CHD prevalence among live births, stillbirths, and terminated pregnancies in an entire nation over a period of 16 years. Methods: Information on all births in the Medical Birth Registry of Norway, 1994-2009, was updated with information on CHD from the hospitals' Patient Administrative Systems, the National Hospital's clinical database for children with heart disease, and the Cause of Death Registry. Individuals with heart defects were assigned specific cardiac phenotypes. Results: Among 954,413 births, 13,081 received a diagnosis of CHD (137.1 per 10,000 births, 133.2 per 10,000 live births). The prevalence per 10,000 births was as follows: heterotaxia, 1.6; conotruncal defects, 11.6; atrioventricular septal defects, 5.6; anomalous pulmonary venous return, 1.1; left outflow obstructions, 8.7; right outflow obstructions, 5.6; septal defects, 65.5; isolated patent ductus arteriosus, 24.6; and other specified or unspecified CHD, 12.7. Excluding preterm patent ductus arteriosus, the CHD prevalence was 123.4 per 10,000; per year, the prevalence increased with 3.5% (95% CI 2.5-4.4) in 1994-2005 and declined with 9.8%(−16.7 to −2.4) from2005 onwards. SevereCHDprevalencewas 30.7 per 10,000; per-year increase was 2.3% (1.1-3.5) in 1994-2004, and per-year decrease was 3.4% (−6.6 to −0.0) in 2004-2009. Numbers included severe CHD in stillbirths and terminated pregnancies. Conclusions: The birth prevalence of CHD declined from around 2005. Specifically, the prevalence of severe CHD was reduced by 3.4% per year from 2004 through 2009. (Am Heart J 2014;168:956-64.) publishedVersion

Background.A congenital heart defect (CHD) can increase the risk of mental health problems in affected children and their parents. The extent to which risk factors for these problems are shared in families or are specific to the individual family member is unclear.Method.Prospective data from the Norwegian Mother and Child Cohort Study (MoBa; n = 93 009) were linked with a nationwide CHD registry, and 408 children with CHD were identified. Mothers' reports on child internalizing problems and their own distress were assessed by questionnaires at child ages 6, 18 and 36 months. A structural model was applied to distinguish between familial (shared) factors and individual-specific factors for mental health problems.Results.CHD was a substantial risk factor for problems in children and their mothers at all time points. CHD contributed on average 31% and 39% to the variance in children's and mothers' problems respectively. Both shared familial and individual-specific factors unique to CHD families contributed to risk for mental health problems. Whereas individual-specific risk factors contributed to the stability of problems in mothers, the effect of these factors lasted only a short time in children. Mutual influences over time were found between the mother's and the child's mental health at 18 and 36 months.Conclusions.The burden of CHD in a child is shared between family members but is also specific to the individual. This study points to a need for both an individual and a family-based approach to provide psychological support to children with CHD and their parents.

Aims Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015. Methods and results This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7 ± 15.4 years; 36% male) from 13 countries were included. Cause-specific mortality was examined before and after July 2006, ‘early’ and ‘late’, respectively. Over a median follow-up of 6.1 years (interquartile range 2.1–21.5 years) 558 deaths were recorded; cause-specific mortality was identified in 411 (74%) cases. Leading causes of death were heart failure (34%), infection (26%), sudden cardiac death (10%), thromboembolism (8%), haemorrhage (7%), and peri-procedural (7%). Heart failure deaths increased in the ‘late’ relative to the ‘early’ era (P = 0.032), whereas death from thromboembolic events and death in relation to cardiac and non-cardiac procedures decreased (P = 0.014, P = 0.014, P = 0.004, respectively). There was an increase in longevity in the ‘late’ vs. ‘early’ era (median survival 52.3 vs. 35.2 years, P Conclusion The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the ‘late’ era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly needed.

OBJECTIVE: Improved diagnostic tools, timely closure of the shunt and a better understanding of the complexity of Eisenmenger syndrome (ES) have led to improved care and treatment in tertiary centres. These may have decreased the incidence of ES and improved survival of patients with ES, although evidence is still lacking. The aim of this study was to investigate temporal changes in incidence, prevalence and mortality in patients with ES for 35 years in the Nordic region.METHODS: This was a retrospective population-based study including 714 patients with ES. Survival analysis was performed based on all-cause mortality and accounting for immortal time bias.RESULTS: The incidence of ES decreased from 2.5/million inhabitants/year in 1977 to 0.2/million inhabitants/year in 2012. Correspondingly, prevalence decreased from 24.6 to 11.9/million inhabitants. The median survival was 38.4 years, with 20-year, 40-year and 60-year survival of 72.5%, 48.4%, and 21.3%, respectively. Complex lesions and Down syndrome were independently associated with worse survival (HR 2.2, pCONCLUSIONS: The incidence and prevalence of ES in the Nordic region have decreased markedly during the last decades. Furthermore, the median age at death increased throughout the study period, indicating prolonged life expectancy in the ES population. However, increasing age represents decreased incidence, rather than improved survival. Nonetheless, longevity with ES is still shorter than in the background population.

We determined the incidence, type, and severity of complications after cardiac catheterisation in children with heart disease in Norway, and we present the results in terms of the International Paediatric and Congenital Cardiac Code (IPCCC) nomenclature for complications. All paediatric cardiac catheterisations in Norway are performed in one clinical centre. All procedures performed during a 5-year period beginning in 2010 were prospectively registered, and medical records for cases with complications were reviewed to confirm the event and to re-classify the type, severity, and attributability of the complication according to the IPCCC nomenclature. Univariate and multivariate analyses were performed to identify possible risk predictors. A total of 1318 catheterisations performed on 941 patients were included in the present study, of which 68% were interventional. The complication and major complication rates were 5.5 and 1.4%, respectively. Trauma to the vessels or the myocardium, haemodynamic adverse events, and arrhythmias were the most common types of complications. In the multivariate model, weight

Patent ductus arteriosus (PDA) in preterm infants is a controversial topic in the management of preterm neonates. There are no generally accepted guidelines for diagnosis, treatment, and follow-up of PDA, and few publications have covered the whole topic or have been conclusively summarized to give a proper direction for the treating physician. Major issues remain to be clarified, both with respect to diagnosis and treatment. The definition of hemodynamic significance varies because of different use of echocardiographic criteria and uncertainty about the role of biomarkers. The detailed risks and benefits of available treatment alternatives are still under investigation. There has been a general shift in the management of PDA in preterm neonates from the "aggressive approach" to a more "conservative approach," but the effects of this strategy on morbidity in a longer time perspective are not fully known. An individualized therapeutic strategy with special emphasis on identification of hemodynamically significance seems to be the way forward. In this review we put forward the scientific background in favor of a seemingly growing body of evidence against active treatment, but we raise caution against shying away from all forms of treatment or instituting them too late. Finally, we try to integrate the current knowledge into suggestions for the management of PDA in premature infants.

While children with chronic illness can experience many physical, functional, and psychosocial symptoms and problems, they do not have the same verbal skills as adults to communicate about distressing symptoms, leaving them at particular risk that their problems remain under-diagnosed and treated. This study investigated the effects of an interactive communication tool for children with heart disease, called Sisom, on patient-provider communication in pediatric cardiology out-patient consultations. Fourty-two children aged 7–13 diagnosed with heart disease used Sisom in preparation for their consultation. The resulting summaries of the children's perceived problems were shared with their nurses and physicians for use during the consultation. A control group, matched on gender and age (n = 42), received usual care. All consultations were video-taped and analyzed with the Roter Interaction Analysis System. Compared to the control group, almost twice as many symptoms and problems were addressed when Sisom wa...

Objective To assess the level of partner relationship satisfaction among mothers of children with different severity of congenital heart defects (CHD) compared with mothers in the cohort. Methods Mothers of children with mild, moderate, or severe CHD (n = 182) and a cohort of mothers of children without CHD (n = 46,782) from the Norwegian Mother and Child Cohort Study were assessed at 5 time points from pregnancy to 36 months postpartum. A 5-item version of the Relationship Satisfaction scale was used, and relevant covariates were explored. Results The trajectories of relationship satisfaction among mothers of children with varying CHD severity did not differ from the trajectories in the cohort. All women in the cohort experienced decreasing relationship satisfaction from 18 months after delivery up to 36 months after delivery. Conclusions Having a child with CHD, regardless of severity, does not appear to exacerbate the decline in relationship satisfaction.

Objective: To investigate the association between pregestational or gestational diabetes and offspring risk of congenital heart defects and the association between large-for-gestational-age birth weight and risk of cardiac defects in offspring of diabetic women. Methods: Information on pregestational and gestational diabetes, cardiac defects, and birth weight among all births in Norway in 1994–2009 was ascertained from the Medical Birth Registry of Norway, national health registries, and the Cardiovascular Disease in Norway project. The relative risk (RR) compared offspring risk of cardiac defects for maternal diabetes with offspring risk in nondiabetic mothers adjusted for year of birth, maternal age, and parity. Results: Among 914,427 births (live births, stillbirths, terminated pregnancies), 5,618 (0.61%) were complicated by maternal pregestational diabetes and 9,726 (1.06%) by gestational diabetes. Congenital heart defects were identified in 10,575 offspring. The prevalence of cardiac defects differed between groups: 344 of 10,000 births to women with pregestational diabetes, 172 of 10,000 to women with gestational diabetes, and 114 of 10,000 in women without diabetes (adjusted RRs 2.92, 95% confidence interval [CI] 2.54–3.36 and 1.47, 95% CI 1.26–1.71). During the study period, the adjusted RRs for congenital heart defects did not change. The risk of cardiac defects in neonates very large for gestational age (birth weight greater than 3 standard deviations above the mean) was compared with neonates with birth weight appropriate for gestational age. For pregestational diabetes, the prevalences of offspring cardiac defects were 561 compared with 248 per 10,000 births (adjusted RR 2.23, 95% CI 1.39–3.59) and for gestational diabetes 388 compared with 132 per 10,000 (adjusted RR 2.73, 95% CI 1.53–4.85). Conclusion: The increased risk of having a child with a congenital heart defect has not changed for diabetic women in Norway since 1994. Among women with pregestational or gestational diabetes, having a large-for-gestational-age neonate was associated with a two- to threefold increased risk of cardiac defects compared with neonates with normal birth weight. acceptedVersion

The objective of this study was to investigate mechanisms of exercise-induced pulmonary hypertension in patients with congenital cardiac septal defects. This was a randomized, placebo controlled, crossover drug trial in a single national pediatric cardiology centre that performs congenital cardiac defect surgery. There were 14 patients with cardiac septal defects and known exercise-induced pulmonary hypertension. The intervention consisted of 50 mg oral sildenafil versus placebo. Measurements included supine bicycle exercise echocardiography and oxygen uptake. The outcome measure was right-ventricular systolic pressure as estimated by Doppler tracings of tricuspid regurgitant jet as well as systolic and diastolic longitudinal myocardial velocities by color tissue Doppler echocardiography. Sildenafil did not change exercise right-ventricular systolic pressure during exercise; however, decreased systemic systolic pressure was seen. Enhanced biventricular longitudinal function with sildenafil compared with placebo was indicated by greater tissue Doppler velocities and displacement measurements during exercise. Finally, a less steep increase of right-ventricular pressure during exercise was associated with greater left-ventricular diastolic myocardial tissue Doppler velocity. Exercise-induced pulmonary hypertension in cardiac septal defects does not seem to have a pulmonary vasoconstrictive component, but it may be related to left-ventricular filling pressure. Furthermore, sildenafil improved biventricular systolic performance in this patient group, possibly related to decreased systemic afterload.

BackgroundVentricular septal defects (VSDs) are the most common congenital heart defects (CHDs). Previous studies indicate an increased risk of endocarditis, aortic regurgitation, left ventricular outflow tract obstructions, pulmonary hypertension, arrhythmias and sudden death in patients with isolated VSDs. The present nationwide cohort study reports mortality and cardiac complications requiring hospitalisation or intervention in children with isolated VSDs.Methods and resultsMedical information concerning all 943 871 live births in Norway in 1994–2009 was retrieved from the Medical Birth Registry of Norway, the Cardiovascular Disease in Norway project, the Oslo University Hospital's Clinical Registry of Congenital Heart Defects and the Norwegian Cause of Death Registry. Isolated VSDs were identified in 3495 children without known chromosomal aberrations or extracardiac malformations. Surgical or catheter-based treatment of VSD was performed in 181 (5.2%) cases. Twelve (0.3%) children with VSDs died before 2013. There was no operative mortality, and no excess mortality in children with isolated VSDs compared with children without VSDs (adjusted HR 0.8 (0.5 to 1.4), p=0.48). The following conditions were recorded as possible cardiac complications of the VSDs: endocarditis in 3 children (0.9‰), aortic regurgitation in 12 children (3.4‰), left ventricular outflow tract obstructions in no children (0.0‰), pulmonary hypertension in 1 child (0.3‰) and arrhythmias in 16 children (4.6‰).ConclusionsThe entire group of children with isolated VSDs had a favourable prognosis without excess mortality. Cardiac complications requiring hospitalisation or intervention, including endocarditis, aortic regurgitation, left ventricular outflow tract obstructions, pulmonary hypertension and arrhythmias, were infrequent during childhood.Trial registration numberNCT02026557.

The aetiology of congenital heart defects (CHD) is mostly unknown, but maternal factors may modify the infant risk of CHD. We investigated the association between maternal preeclampsia and offspring risk of severe CHD in a nation-wide cohort study.Information on all births registered in the Medical Birth Registry of Norway, 1994-2009, was completed with information on CHD diagnoses from national health registries and the Cardiovascular Diseases in Norway Project (CVDNOR).Among 914 703 singleton births without chromosomal abnormalities, 32 864 (3.6%) were born after a pregnancy with preeclampsia. The preeclampsia was diagnosed before the 34th week of pregnancy (early-onset preeclampsia) in 2618 (8.0% of preeclamptic pregnancies). CHDs were diagnosed in 10 691 infants; of these, 2473 had severe CHD. The risk of severe CHD was compared between births with and without maternal preeclampsia and estimated with binomial log-linear regression. When adjusting for year of birth, maternal age, parity, and pregestational diabetes, the risk ratio (RR) for severe CHD in offspring of mothers with any preeclampsia was 1.3 [95% confidence interval (CI) 1.1, 1.5], and in pregnancies with early-onset preeclampsia, the RR was 2.8 (95% CI 1.8, 4.4). The association between early-onset preeclampsia and specific types of severe CHD was stronger for atrioventricular septal defects (AVSD), with adjusted RR 13.5 (95% CI 6.8, 26.8).Early-onset preeclampsia was strongly associated with infant risk of severe CHD, specifically; the risk of AVSD was 15-fold higher if the mother was diagnosed with early-onset preeclampsia, suggesting common aetiological factors for early-onset preeclampsia and erroneous fetal heart development.