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We report our experience in simultaneously implanting multiple stents and valves mounted on a single balloon before and during transcatheter pulmonary valve placement.Heterogeneity and complexity of the right ventricular outflow tract (RVOT) may complicate stent deployment when preparing a landing zone for transcatheter pulmonary valve implantation.Retrospective analysis of patients from Children's Hospital of Colorado, USA; and Oslo University Hospital, Norway, undergoing transcatheter pulmonary valve replacement that had at least two stents mounted on a single balloon, deployed in the RVOT.Over a 42-month period, a total of 50 subjects from the two centers met inclusion criteria for the study. Subjects were predominantly male (58%), and the median age was 17 years (4-78 years). In six subjects (12%), there was need for prestenting with use of the double or triple stent piggyback technique. Forty subjects (80%) had a Melody ™ TPV implanted. In 45 cases (90%), one or more stents were mounted over the pulmonary valve using its delivery system, either the Ensemble for the Melody™ TPV or the Edwards Commander for the SAPIEN 3 THV. Thirty-seven subjects (74%) had one stent mounted and eight subjects (16%) had two stents mounted over the pulmonary valve for simultaneous deployment. No complications related to this technique were reported.The piggyback technique aims to simplify and facilitate adequate conduit preparation and valve insertion by minimizing manipulation across the outflow tract and decreasing the risk of stent distortion, misalignment, and embolization.

There is a risk of gaseous and solid micro-embolus formation during transcatheter cardiac interventions and surgery in children with congenital heart disease (CHD). Our aim was to study the burden of high-intensity transient signals (HITS) during these procedures in infants. We used a novel color M-mode Doppler (CMD) technique by NeoDoppler, a non-invasive ultrasound system based on plane wave transmissions for transfontanellar continuous monitoring of cerebral blood flow in infants. The system displays CMD with 24 sample volumes and a Doppler spectrogram. Infants with CHD undergoing transcatheter interventions (n = 15) and surgery (n = 13) were included. HITS were manually detected based on an “embolic signature” in the CMD with corresponding intensity increase in the Doppler spectrogram. Embolus-to-blood ratio (EBR) defined HITS size. A total of 1169 HITS with a median EBR of 9.74 dB (interquartile range [IQR]: 5.10–15.80 dB) were detected. The median number of HITS in the surgery group was 45 (IQR: 11–150), while in the transcatheter group the median number was 12 (IQR: 7–24). During cardiac surgery, the highest number of HITS per hour was seen from initiation of cardiopulmonary bypass to aortic X-clamp. In this study we detected frequent HITS and determined the feasibility of using NeoDoppler monitoring for HITS detection.

Background Heart transplantation (HTx) surgically denervates the heart. We examined indices of sinoatrial reinnervation, with emphasis on (1) the occurrence and timing of parasympathetic reinnervation, and (2) the consequences of reinnervation for heart rate (HR) responsiveness and arterial baroreceptor sensitivity. Methods Fifty HTx recipients were prospectively followed for 36 months after surgery. Hemodynamic variables and heart rate variability were continuously recorded at supine rest, 60 degrees head-up-tilt, during the Valsalva maneuver and during handgrip isometric exercise. Results Suggesting parasympathetic reinnervation: at baseline rest, root of the mean squared differences of successive RR intervals increased from median 3.9(5.9) to 7.1(5.1) ms (p < 0.001); high-frequency power increased from 4.0(12) to 5.7(18.9) ms2 (p = 0.018); and baroreceptor sensitivity increased from 0.04(0.36) to 1.3(2.4) ms/mmHg (p < 0.001). Suggesting sympathetic reinnervation: at baseline rest low-frequency power increased from 0.49(2.5) to 7.5(18.1) ms2 (p < 0.001); and HR responses to sympathetic stimulation during (1) head-up tilt increased from 1.9(4.2) to 9.1(8.2) bpm (p < 0.001), (2) Valsalva increased from 1.6(1.4) to 8.3(10.8) bpm (p < 0.001) and (3) handgrip increased from 0.3(0.6) to 1.9(5.1) bpm (p < 0.001). Signs of sympathetic reinnervation emerged within 6 months, while signs of parasympathetic reinnervation emerged by 24 months. Conclusions Root of the mean squared differences of successive RR intervals, high-frequency and low-frequency variability, HR responsiveness and arterial baroreflex sensitivity all increased after HTx, suggesting functional parasympathetic and sympathetic sinoatrial reinnervation. Accordingly, the pathological regulatory state present in heart transplant recipients, which is responsible for a host of functional and clinical abnormalities, is being partially offset over time by restored autonomic control of the heart in many heart transplant recipients.

Background Heart transplantation (HTx) surgically transects all connections to the heart, including the autonomic nerves. We prospectively examined signs, timing and consequences of early sympathetic and parasympathetic sinoatrial reinnervation, as well as explored indirect evidence of afferent cardiopulmonary reinnervation. Methods Fifty HTx recipients were assessed at 2.5, 6 and 12 months after HTx. For comparison, 50 healthy controls were examined once. Continuous, noninvasive recordings of hemodynamic variables and heart rate variability indices were carried out at supine rest, 0.2 Hz controlled breathing, 60 degrees head-up-tilt, during the Valsalva maneuver and during handgrip isometric exercise. Results In HTx recipients, supine low-frequency heart rate variability gradually increased; supine high-frequency variability did not change; heart rate variability indices during controlled breathing remained unaltered; heart rate responses during tilt and isometric exercise gradually increased; the tachycardia response during Valsalva maneuver increased while the bradycardia response remained unchanged; and indices of baroreflex sensitivity improved. Responses remained low compared to healthy controls. A negative correlation between indices of preload and heart rate response during head-up tilt emerged at 12 months. Conclusions Results suggest that sympathetic reinnervation of the sinoatrial node starts within 6 months after HTx and strengthens during the first year. No evidence of early parasympathetic reinnervation was found. Indirect signs of afferent reinnervation of cardiopulmonary low-pressure baroreceptors emerged at 12 months. Better sympathetic sinoatrial control improved heart rate responsiveness to orthostatic challenge and isometric exercise, as well as heart rate buffering of blood pressure fluctuations.

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

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.

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.

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

Objective We investigated bone mineral density (BMD) at different ages after the Fontan completion, and we evaluated the relationship between BMD, vitamin D levels, and pertinent patient variables. Methods A cross-sectional sample of 64 patients was examined with dual-energy X-ray absorptiometry (DXA) scans to determine BMD. Of these patients, 24 were also examined with BoneXpert software to determine bone mass density (BMX), expressed as the bone health index (BHI). Blood samples from all patients were analyzed. Patients were divided into three different age groups; A: 4-9 years old (n = 22), B: 10-15 years old (n = 21), and C: 16-18 years old (n = 21). Results Overall, BMD z scores were (mean ± SD): -1.0 ± 1.3 for the lumbar spine and -0.2 ± 1.2 for the total body. Groups B and C had significantly lower z score values compared to group A. Of patients in group C, 35% had z score values ≤-2 SD of the mean of the healthy population. There was no difference related to systemic ventricular anatomy (left or right); however, patients with lateral tunnels had lower BMD than patients with extra cardiac conduits. Overall, the BHI z score was (mean ± SD): -1.2 ± 0.9, but low BMX did not correlate with low BMD. The 25-hydroxy vitamin D level was 58 ± 30 nmol/L. Vitamin D levels decreased with age: in group C, 33.3% of patients exhibited vitamin D deficiencies. Vitamin D levels were not correlated with bone mineral densities. Conclusion BMD levels decreased with age in patients with Fontan circulation. Different bone components were involved. Vitamin D levels also decreased with age, but they were not consistently associated with bone mineral densities. The single factor most predictive of low BMD was a lateral tunnel Fontan, compared to an extra cardiac Fontan.

Heart transplantation (HTx) implies denervation of afferent neural connections. Reinnervation of low-pressure cardiopulmonary baroreceptors might impact the development and treatment of hypertension, but little is known of its occurrence. The present prospective study investigated possible afferent reinnervation of low-pressure cardiopulmonary baroreceptors during the first year after heart transplantation.A total of 50 heart transplant recipients (HTxRs) were included and were evaluated 7-12 weeks after transplant surgery, with follow-up 6 and 12 months later. In addition, a reference group of 50 healthy control subjects was examined once. Continuous, non-invasive recordings of cardiovascular variables were carried out at supine rest, during 15 min of 20° head-up tilt, during Valsalva maneuver and during 1 min of 30% maximal voluntary handgrip. In addition, routine clinical data including invasive measurements were used in the analyses.During the first year after HTx, the heart rate (HR) response to 20° head-up tilt partly normalized, a negative relationship between resting mean right atrial pressure and HR tilt response developed, low-frequency variability of the RR interval and systolic blood pressure at supine rest increased, and the total peripheral resistance response to Valsalva maneuver became stronger.Functional assessments suggest that afferent reinnervation of low-pressure cardiopulmonary receptors occurs during the first year after heart transplantation, partially restoring reflex-mediated responses to altered cardiac filling.

Background Children with Fontan circulation are at risk of developing hepatic fibrosis/cirrhosis. Reliable noninvasive monitoring techniques are lacking or under development. Objective To investigate surrogate indicators of hepatic fibrosis in adolescents with Fontan circulation by evaluating hepatic magnetic resonance (MR) T1 mapping and extracellular volume fraction measurements compared to US shear-wave elastography. Materials and methods We analyzed hepatic native T1 times and extracellular volume fractions with modified Look-Locker inversion recovery. Liver stiffness was analyzed with shear-wave elastography. We compared results between 45 pediatric patients ages 16.7±0.6 years with Fontan circulation and 15 healthy controls ages 19.2±1.2 years. Measurements were correlated to clinical and hemodynamic data from cardiac catheterization. Results MR mapping was successful in 35/45 patients, revealing higher hepatic T1 times (774±44 ms) than in controls (632±52 ms; PPPP=0.007). Native T1 was not correlated with elastography in patients (r=0.2, P=0.1) or controls (r = −0.3, P=0.3). Extracellular volume fraction was correlated with elastography in patients (r=0.5, P=0.005) but not in controls (r=0.2, P=0.6). Conclusion Increased hepatic MR relaxometry and shear-wave elastography values in adolescents with Fontan circulation suggested the presence of hepatic fibrosis or congestion. Central venous pressure was related to T1 times. Changes were detected differently with MR relaxometry and elastography; thus, these techniques should not be used interchangeably in monitoring hepatic fibrosis.

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.

Purpose Heart transplantation causes denervation of the donor heart, but the consequences for cardiovascular homeostasis remain to be fully understood. The present study investigated cardiovascular autonomic control at supine rest, during orthostatic challenge and during isometric exercise in heart transplant recipients (HTxR). Methods A total of 50 HTxRs were investigated 7–12 weeks after transplant surgery and compared with 50 healthy control subjects. Continuous, noninvasive recordings of cardiovascular variables were carried out at supine rest, during 15 min of 60° head-up tilt and during 1 min of 30% of maximal voluntary handgrip. Plasma and urine catecholamines were assayed, and symptoms were charted. Results At supine rest, heart rate, blood pressures and total peripheral resistance were higher, and stroke volume and end diastolic volume were lower in the HTxR group. During tilt, heart rate, blood pressures and total peripheral resistance increased less, and stroke volume and end diastolic volume decreased less. During handgrip, heart rate and cardiac output increased less, and stroke volume and end diastolic volume decreased less. Orthostatic symptoms were similar across the groups, but the HTxRs complained more of pale and cold hands. Conclusion HTxRs are characterized by elevated blood pressures and total peripheral resistance at supine rest as well as attenuated blood pressures and total peripheral resistance responses during orthostatic challenge, possibly caused by low-pressure cardiopulmonary baroreceptor denervation. In addition, HTxRs show attenuated cardiac output response during isometric exercise due to efferent sympathetic denervation. These physiological limitations might have negative functional consequences.

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

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.

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

Objectives: From a population of 90 patients after pulmonary valve replacement with a biological valve (Carpentier-Edwards Perimount valve), 56 of 80 available patients were examined five years after surgery. Background: Pulmonary valve replacement is needed in many patients with congenital heart disease. Homografts have limited availability and predictable degeneration, and mechanical valves require anticoagulation. No superiority of one kind of pulmonary valve replacement has been shown. Biological valves that are readily available are being used and evaluated in increasing numbers. Methods: In this cross-sectional study, five years following surgery, data were gathered from hospital charts, echocardiography, stress echocardiography, magnetic resonance imaging, and exercise testing. Results: In 90 patients, there were three new valve replacements, one early cardiac death, and four late noncardiac deaths. Echocardiographic assessment of the study group showed pulmonary Doppler velocities (m/s) before, after operation, and at five-year follow-up of 2.8 ± 1.1, 1.6 ± 0.4, and 2.3 ± 0.7, respectively. The assessed insufficiencies (0-3) at the same times were 2.3 ± 1.0, 0.3 ± 0.4, and 1.1 ± 0.8. Maximal oxygen uptake increased from 65.6% ± 10.1% to 77.1% ± 18.2% of predicted and QRS width increased by 7 ± 23ms. Valve degeneration could be associated with young age but not with diagnosis or valve size. Conclusion: In our study, the biological valve in the pulmonary position showed excellent mid-term results with few reoperations, low gradients, and mild to moderate insufficiency. Oversizing, in contrast to young age, was not a risk factor for valve degeneration. In younger patients, this allows later percutaneous replacement, reducing the need for further surgery. However, longer follow-up is needed.

In a retrospective study we assessed surgical results following right ventricular to pulmonary artery connection repair or replacement at a medium of 2.4 years (0-8) follow-up. Data were retrieved from hospital charts.Three hundred and sixty five operations were performed in 286 patients in eight years starting in 2000 using different surgical methods. Homografts and Monocusps had a more than 50% significantly lower risk for reoperation than Contegra or bicuspid valves (p0.01). Data for infants and older children and grown ups were analysed separately. In the infant group no significant difference between the different methods (homograft, Contegra and Monocusp) was detected. In older patients, the Perimount valves performed extremely well with no need for reoperation after 2.5 years of follow-up. Perimount valves and homografts performed better than other solutions (p = 0.01).Although the follow-up for the Perimount valves was short, they are promising and need to be followed long-term. The homograft and the Monocusp remain valuable choices.

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 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.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.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.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.

Aims Congenital heart defects (CHDs) are the most common birth defects and are an important cause of death in children. The fear of sudden unexpected death has led to restrictions of physical activity and competitive sports. The aim of the present study was to investigate the rate of sudden unexpected deaths unrelated to surgery in children 2–18 years old with CHDs and, secondarily, to determine whether these deaths were related to cardiac disease, comorbidity, or physical activity. Methods and results To identify children with CHDs and to determine the number of deaths, data concerning all 9 43 871 live births in Norway in 1994–2009 were 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, and information for the deceased children was retrieved from medical records at Norwegian hospitals. Among 11 272 children with CHDs, we identified 19 (0.2%) children 2–18 years old who experienced sudden unexpected deaths unrelated to cardiac surgery. A cardiac cause of death was identified in seven of these cases. None of the children died during physical activity, whereas two children survived cardiac arrest during sports. Conclusion Sudden unexpected death was infrequent among children with CHDs who survived 2 years of age. Comorbidity was common among the children who died. This study indicates that sudden unexpected death in children with CHDs rarely occurs during physical activity.

Objective Percutaneous pulmonary valve implantation is an alternative to open heart surgery in selected patients with pulmonary outflow tract disorder. The technique may reduce the number of open-chest surgeries in these patients. This study was conducted to understand how the patients and their next-of-kin experienced this new treatment option. Design Qualitative explorative design with individual in-depth interviews. Setting Oslo University Hospital, the only cardiac centre in Norway offering advanced surgical and interventional treatment to patient with congenital heart defects, serving the whole Norwegian population. Participants During a 2-year period a total of 10 patients, median age 17 (7–30) and 18 next-of-kin were consecutively selected for individual in-depth interviews 3–6 months after the pulmonary valve implantation. The verbatim transcripts were analysed using a phenomenological methodology. Results Patients emphasised the importance of regaining independence and taking control of daily life shortly after the new interventional treatment. Renewed hope towards treatment options was described as ‘a light in the tunnel’. Next-of-kin emphasised the importance both for the patient and their family of resuming normal life quickly after the procedure. The physical burden was experienced as minor after the minimally invasive intervention, compared to their previous experience with surgical procedures. Main outcome measure The importance of maintaining normality in everyday life for a good family function. Conclusions The repeated surgeries during infancy and adolescence of patients with congenital heart disease represent a heavy burden both for the patient and their family. All families especially emphasised the importance of resuming normal life quickly after each procedure. The novel technique of pulmonary valve implantation is thus a favourable approach because of minor interference in daily life.

Background Flow visualization before transcatheter atrial septal defect (ASD) closure is essential to identify the number and size of ASDs and to map the pulmonary veins (PV). Previous reports have shown improved visualization of ASD and PV using blood flow imaging (BFI), which supplements color Doppler imaging (CDI) with angle-independent information of flow direction. In this study, we compared transesophageal BFI with the current references in ASD sizing (balloon stretched diameter, BSD) and PV imaging (pulmonary angiography). Methods In this prospective study, 28 children were examined with transesophageal echocardiography (TEE) including BFI of the secundum ASD and the PV before interventional ASD closure. The maximum ASD diameter measured with BFI by 4 observers was compared to the corresponding BSD and CDI measurements. The repeatability of the BFI measurements was calculated as the residual standard deviation. BFI of the PV was compared to PV angiography. Results The mean maximum diameter measured by BFI was 12.1 mm (±SD 2.4 mm). The corresponding BSD and CDI measurements were 15.9 mm (±SD 3.0 mm) and 11.8 mm (±SD 2.5 mm), respectively. The residual standard deviation was 1.2 mm. Compared to PV angiography, the sensitivity of BFI in detecting the correct entry of the PV was 0.96 (95% CI: 0.82–1.0). Conclusion Transesohageal echocardiography with BFI of the PV agreed well with pulmonary angiography. BFI had lower estimates for ASD size than BSD, but with acceptable 95% limits of agreement. The repeatability of the BFI measurements was close to the inherent ultrasound measurement error.

Introduction: The aim of this prospective study was to determine the frequency and composition of cerebral microemboli in a paediatric population during transcatheter atrial septal defect closure. Methods: Multi-frequency transcranial Doppler was used to detect microembolic signals in the middle cerebral artery of 24 patients. Embolic signals were automatically identified and differentiated according to their composition, gaseous or solid. The procedure was divided into five periods: right cardiac catheterisation; left cardiac catheterisation; pulmonary angiography; balloon sizing; and device placement. Results: Microemboli were detected in all patients. The median number of signals was 63 and over 95% gaseous. The total number of microembolic signals detected during two periods – balloon sizing and sheath placement and device placement – was not significantly different (median: 18 and 25, respectively) but was significantly higher than each of the other three periods (pConclusion: This is the first study to investigate the timing and composition of cerebral microemboli in a paediatric population during cardiac catheterisation. Microembolic signals were related to specific catheter manipulations but were not associated with fluoroscopic time or duration of procedure.

© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. OBJECTIVES: Today, both surgical and percutaneous techniques are available for pulmonary valve implantation in patients with right ventricle outflow tract obstruction or insufficiency. In this controlled, non-randomized study the hospital costs per patient of the two treatment options were identified and compared. METHODS: During the period of June 2011 until October 2014 cost data in 20 patients treated with the percutaneous technique and 14 patients treated with open surgery were consecutively included. Two methods for cost analysis were used, a retrospective average cost estimate (overhead costs) and a direct prospective detailed cost acquisition related to each individual patient (patient-specific costs). RESULTS: The equipment cost, particularly the stents and valve itself was by far the main cost-driving factor in the percutaneous pulmonary valve group, representing 96% of the direct costs, whereas in the open surgery group the main costs derived from the postoperative care and particularly the stay in the intensive care department. The device-related cost in this group represented 13.5% of the direct costs. Length-of-stay-related costs in the percutaneous group were mean $3885 (1618) and mean $17 848 (5060) in the open surgery group. The difference in postoperative stay between the groups was statistically significant (P ≤ 0.001). CONCLUSIONS: Given the high postoperative cost in open surgery, the percutaneous procedure could be cost saving even with a device cost of more than five times the cost of the surgical device.

Background: Oxygen supplementation is still part of international resuscitation protocols for premature children. Mechanisms for tissue damage by hypoxia/ischemia in the extreme premature involve inflammation. Aim and method: To study cerebral inflammation after hypoxia/ischemia and oxygen treatment in the premature, we measured NF-κB activity in 5-day-old transgenic reporter mice in response to experimental hypoxia/ischemia. Results were correlated to cerebral histological evaluation and plasma cytokine levels. A treatment strategy with the antioxidant tempol was tested. Results: One day after hypoxia/ischemia NF-κB activation was increased compared to controls [mean difference: 10.6±4.6% (P=0.03)]. Exposure to 100% oxygen after hypoxia/ischemia further increased NF-κB activation compared to hypoxia/ischemia alone [mean difference: 15.0±5.5% (P=0.01)]. Histological changes in the brain were positively correlated with NF-κB activity (P Conclusion: Cerebral inflammatory response after hypoxia/ischemia in a mouse model with immature brain development corresponding to human prematurity prior to 32 weeks’ gestation was influenced by administration of oxygen. Tempol treatment attenuated inflammation but did not reduce the extent of histological cerebral damage.

Percutaneous catheter-based implantation of artificial heart valves is a new technique that may supplement surgery and which may be used more in the future. We here report our first experience with implantation of artificial pulmonary valves in children with congenital heart defects.Eligible patients were those with symptoms of heart failure combined with stenosis and/or insufficiency in an established artificial right ventricular outflow tract. The valve was inserted through a catheter from a vein in the groin or neck. Symptoms, echocardiography, invasive measurements and angiography were assessed for evaluation of treatment effect. Our treatment results are reported for the period April 2007-September 2009.Ten patients (seven men and three women, median age 17 years) were assessed. The procedure reduced pressure in the right ventricle (p = 0.008) and resolved the pulmonary insufficiency in all patients. The median time in hospital was two days. No patients had complications that were directly associated with the implantation procedure. One patient developed a pseudoaneurysm in the femoral artery, another had a short-lasting fever two days after the procedure and one patient experienced a stent fracture that required surgery 9 months after the implantation. After 6 months all patients had a reduced pressure gradient in the right ventricular outflow tract (p = 0.008), the pulmonary insufficiency had improved (p = 0.006) and they all reported improval of symptoms. These results persisted for at least 24 months for the four patients who were monitored until then.Percutaneous catheter-based implantation of artificial pulmonary valves improves hemodynamics in the right ventricle of selected patients with congenital heart defects. A randomized controlled study should be undertaken to provide a stronger evidence-base for usefulness of this procedure.

Background Patients with Jervell and Lange-Nielsen syndrome (JLNS) exhibit severe phenotypes that are characterized by congenital deafness, very long QT intervals, and high risk of life-threatening arrhythmias. Current treatment strategies include high doses of beta-blocker medication, left cardiac sympathetic denervation, and ICD placement, which is challenging in young children. Objective The purpose of this study was to evaluate the safety and effect of pacing in addition to beta-blocker treatment in children with JLNS. Methods All genetically confirmed patients with JLNS born since 1999 in Norway were included in the study. Data on history of long QT syndrome–related symptoms, QT interval, and beta-blocker and pacemaker treatment were recorded. Results A total of 9 patients with QT intervals ranging from 510 to 660 ms were identified. Eight patients developed long QT syndrome–related symptoms, and 1 patient died before diagnosis. The survivors received beta-blocker medication. Seven patients also received a pacemaker; 1 had a ventricular lead and 6 had atrial leads. The patient with the ventricular lead died during follow-up. The 6 patients with atrial leads survived without events at a mean follow-up of 6.9 years after pacemaker implantation. Two patients received prophylactic upgrade to a 2-chamber ICD. Conclusion No arrhythmic events occurred in 6 very young JLNS patients who received atrial pacing in combination with increased doses of beta-blockers during 7-year follow-up. If confirmed in additional patients, this treatment strategy may prevent life-threatening arrhythmias in this high-risk patient group and may act as a bridge to insertion of a 2-chamber ICD when left cardiac sympathetic denervation is not available.