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Aim To study the long‐term outcome after surgery for pulmonary atresia and ventricular septal defect (PA‐VSD), and to determine association between the contribution of major aorto‐pulmonary collateral arteries (MAPCAs) to the pulmonary blood flow, comorbidity and cause of death. Methods Patients who had undergone surgery for PA‐VSD from January 1st 1994 to December 31st 2017 were studied retrospectively. Survival was cross‐checked against the Swedish National Population Register. Results Seventy patients were identified, giving an incidence of 5.3 newborns per 100 000 live births. In 41 patients (59%) the pulmonary blood flow originated from a patent ductus arteriosus (PDA), while 29 patients (41%) had contribution of the pulmonary blood flow from MAPCAs. Extracardiac disease was found in 34 patients (49%), 16 of whom had 22q11‐microdeletion syndrome (23%). Survival at follow‐up was similar in patients with and without MAPCAs (72.4% vs. 75.6%, n.s.), with a median follow‐up time of 14.3 years (3.2–41.8 years). No difference was found in mortality in patients with or without any syndrome or extracardiac disease. Conclusion Long‐term survival did not differ between those with and without MAPCAs and no difference in mortality was seen in patients with and without concomitant extracardiac disease or any kind of syndrome.

Previous research in children and adolescents with congenital heart defects presents contradictory findings concerning their physical activity (PA) level, due to methodological limitations in the PA assessment. The aim of the present cross-sectional study was to compare PA in children and adolescents treated for valvular aortic stenosis with healthy controls using an improved accelerometer method. Seven-day accelerometer data were collected from the hip in a national Swedish sample of 46 patients 6–18 years old treated for valvular aortic stenosis and 44 healthy controls matched for age, gender, geography, and measurement period. Sports participation was self-reported. Accelerometer data were processed with the new improved Frequency Extended Method and with the traditional ActiGraph method for comparison. A high-resolution PA intensity spectrum was investigated as well as traditional crude PA intensity categories. Children treated for aortic stenosis had a pattern of less PA in the highest intensity spectra and had more sedentary time, while the adolescent patients tended to be less physically active in higher intensities overall and with less sedentary time, compared to the controls. These patterns were evident using the Frequency Extended Method with the detailed PA intensity spectrum, but not to the same degree using the ActiGraph method and traditional crude PA intensity categories. Patients reported less sports participation than their controls in both age-groups. Specific differences in PA patterns were revealed using the Frequency Extended Method with the high-resolution PA intensity spectrum in Swedish children and adolescents treated for valvular aortic stenosis.

BackgroundInfants with congenital heart disease (CHD) have an increased risk of morbidity and mortality during a respiratory syncytial virus (RSV) infection. The aim of this study was to estimate the cost-effectiveness of palivizumab as RSV-prophylaxis among infants with CHD, including the effect of delayed heart surgery and asthma.MethodsA simulation model with data from the literature and health care authorities including costs and utilities was developed to estimate costs and health effects over a lifetime for a cohort of CHD infants receiving palivizumab compared to no RSV-prophylaxis.ResultsThe prophylaxis treatment incurred a cost of 3664 EUR per treated infant. However, due to cost-savings from primarily avoiding hospitalizations (5145 EUR/treated infant) and avoiding heart complications due to delayed heart surgery (2082 EUR/treated infant), the RSV-prophylaxis treatment resulted in a total cost-saving of 3833 EUR per treated infant. At the same time, the prophylaxis-treated cohort accumulated more life-years and higher quality of life than the non-prophylaxis cohort.ConclusionThis study confirms that RSV-prophylaxis in severe CHD infants less than one year of age is cost beneficial. Avoiding delayed heart surgeries is an important benefit of prophylaxis and should be taken into consideration.

Objective:This study describes short-term and long-term outcome after treatment of critical valvular aortic stenosis in neonates in a national cohort, with surgical valvotomy as first choice intervention.Methods:All neonates in Sweden treated for critical aortic stenosis between 1994 and 2016 were included. Patient files were analysed and cross-checked against the Swedish National Population Registry as of December 2017, giving complete survival data. Diagnosis was confirmed by reviewing echo studies. Critical aortic stenosis was defined as valvular stenosis with duct-dependent systemic circulation or depressed left ventricular function. Primary outcome was all-cause mortality and secondary outcomes were reintervention and aortic valve replacement.Results:Sixty-one patients were identified (50 boys, 11 girls). Primary treatment was surgical valvotomy in 52 neonates and balloon valvotomy in 6. Median age at initial treatment was 5 days (0–26), and median follow-up time was 10.8 years (0.14–22.6). There was no 30-day mortality but four late deaths. Freedom from reintervention was 66%, 61%, 54%, 49%, and 46% at 1, 5, 10, 15, and 20 years, respectively. Median time to reintervention was 3.4 months (4 days to 17.3 years). Valve replacement was performed in 23 patients (38%).Conclusions:Surgical valvotomy is a safe and reliable treatment in these critically ill neonates, with no 30-day mortality and long-term survival of 93% in this national study. At 10 years of age, reintervention was performed in 54% and at end of follow-up 38% had had an aortic valve replacement.

Aim The aim of the study was to assess the incidence, mortality and morbidity of dilated cardiomyopathy (DCM) and noncompaction of the left ventricle (LVNC) in Swedish children. Methods We reviewed hospital records of all children with dilated cardiomyopathy (DCM) or left ventricular noncompaction cardiomyopathy (LVNC) up to the age of 18 in the healthcare region of western Sweden from 1991 to 2015. Results In total, 69 cases (61% males) were identified. The combined incidence of DCM and LVNC was 0.77 (95% CI 0.59‐0.96) per 100 000 person years. Children were divided into six groups, and their outcomes were analysed depending on their aetiology. Idiopathic DCM was reported in 43%, and familial dilated and left ventricular noncompaction aetiology was present in 32%. DCM due to various diseases occurred in 8%. DCM associated with neuromuscular diseases was present in 16%. The overall risk of death or receiving transplants in children with idiopathic and familial DCM was 30% over the study period, and 21% died in the first year after diagnosis. Conclusion The combined incidence of DCM and LVNC was similar to previous reports. Most children with idiopathic DCM presented during infancy, and mortality was highest during the first year after diagnosis.

Respiratory tract infections (RTI) are common among young children, and congenital heart defect (CHD) is a risk factor for severe illness and hospitalization. This study aims to assess the relative risk of hospitalization due to RTI in winter and summer seasons for different types of CHD. All children born in Sweden and under the age of two, in 2006-2011, were included. Heart defects were grouped according to type. Hospitalization rates for respiratory syncytial virus (RSV) infection and RTI in general were retrieved from the national inpatient registry. The relative risk of hospitalization was calculated by comparing each subgroup to other types of CHD and otherwise healthy children. The relative risk of hospitalization was increased for all CHD subgroups, and there was a greater increase in risk in summer for the most severe CHD. This included RSV infection, as well as RTI in general. The risk of hospitalization due to RTI is greater for CHD children. Prophylactic treatment with palivizumab, given to prevent severe RSV illness, is only recommended during winter. We argue that information to healthcare staff and parents should include how the risk of severe infectious respiratory tract illnesses, RSV and others, is present all year round for children with CHD.

Objective: Fontan surgery is performed in children with univentricular heart defects. Previous data regarding permanent pacemaker implantation frequency and indications in Fontan patients are limited and conflicting. We examined the prevalence of and risk factors for pacemaker treatment in a consecutive national cohort of patients after Fontan surgery in Sweden. Methods: We retrospectively reviewed all Swedish patients who underwent Fontan surgery from 1982 to 2017 (n = 599). Results: After a mean follow-up of 12.2 years, 13% (78/599) of the patients with Fontan circulation had received pacemakers. Patients operated with the extracardiac conduit (EC) type of total cavopulmonary connection had a significantly lower prevalence of pacemaker implantation (6%) than patients with lateral tunnel (LT; 17%). Mortality did not differ between patients with (8%) and without pacemaker (5%). The most common pacemaker indication was sinus node dysfunction (SND) (64%). Pacemaker implantation due to SND was less common among patients with EC. Pacemaker implantation was significantly more common in patients with mitral atresia (MA; 44%), double outlet right ventricle (DORV; 24%) and double inlet left ventricle (DILV; 20%). In contrast, patients with pulmonary atresia with intact ventricular septum and hypoplastic left heart syndrome were significantly less likely to receive a pacemaker (3% and 6%, respectively). Conclusions: Thirteen percent of Fontan patients received a permanent pacemaker, most frequently due to SND. EC was associated with a significantly lower prevalence of pacemaker than LT. Permanent pacemaker was more common in patients with MA, DORV, and DILV. (Less)

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.

OBJECTIVES This study presents short- and long-term follow-up after treatment for isolated valvular aortic stenosis in children with surgical valvotomy as the preferred 1st intervention. METHODS All patients aged 0-18 years treated between 1994 and 2013 at our centre were reviewed regarding the mode of first treatment, mortality, reinterventions and the need for aortic valve replacement. RESULTS A total of 113 patients were identified in local registries. There were 44 neonates, 31 infants and 38 children. The mean follow-up period was 11 years (range 2-22 years). No early deaths and only 2 late deaths were reported. Of the 113 patients, 92 patients had open surgical valvotomy as the 1st intervention. Freedom from reintervention was 80%, 69%, 61%, 57% and 56% at 1, 5, 10, 15 and 20 years, respectively. The main indication for reintervention was valvular stenosis. Freedom from aortic valve replacement was 67%. CONCLUSIONS Surgical valvotomy of aortic stenosis in this long-term follow-up study resulted in no 30-day mortality and

The overall aim was to assess the reliability and accomplish a limited validation of the Pediatric Quality of Life Inventory 3.0 Cardiac Module Scales (PedsQL 3.0), Swedish version, in a sample of Swedish children diagnosed with congenital heart defects (CHD). A secondary aim was to assess whether the children's health-related quality of life (HRQOL) was affected by gender, age, severity of the CHD, surgical intervention and whether the child self-report and parent proxy-report were consistent. A total of 126 families at The Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Gothenburg, participated in the study. The PedsQL Inventory 4.0 Generic Core Scales (PedsQL 4.0) and the PedsQL 3.0 were administered to 94 children (aged 5–18 years) with CHD and 126 parents (of children with CHD aged 2–18 years). The results showed that the internal consistency of the PedsQL 3.0 reached or exceeded Cronbach's alpha values of 0.70 for both child self-report and parent proxy-report. The PedsQL 4.0 and ...

Background Studies suggest that children with congenital heart defects (CHD) are at risk for adverse intellectual functioning. However, factors related to lower intellectual functioning in this group are largely unknown. This study describes intellectual functioning in children with CHD in relation to severity of the heart defect, the child’s age, and the socioeconomic status of the family (SES). Methods Two hundred twenty-eight children treated with surgery or by catheter technique were tested using the Wechsler intelligence scales to determine full scale IQ (FSIQ). FSIQ was then analyzed in relation to age (3-, 5-, 9-, and 15-year olds), severity of the diagnosis (mild, moderate, and severe), and SES (low, medium, and high). The median age was 70 months (5.8 years) with a range of 162 months [30 months (2.5 years) to 192 months (16.0 years)]. Results The total mean score on FSIQ was 100.8 (SD = 14.5). Children with severe CHD had significantly lower FSIQ than children with mild and moderate CHD, and 9- and 15-year olds had significantly lower FSIQ compared to the 3-year olds. Children from families with low SES had significantly lower FSIQ than children from medium SES and high SES families. No interaction between severity of diagnosis, age, and SES was found for FSIQ. Conclusion Eighty-three percent of the children with CHD performed at or above average with respect to FSIQ. SES and severity of diagnosis had significant main effects on FSIQ. These factors should be considered when planning interventions and follow-up programs for children with CHD.

Background— Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. Methods and Results— We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. Conclusions— Preoperative clinical and morphological features are important risk factors for postoperative PVO and survival.

The outcome for all children born in Sweden between January 1, 1980 and December 31, 2008 with ventriculo-coronary arterial communications (VCACs) and pulmonary atresia with intact ventricular septum (PAIVS) was assessed. Fifty-one subjects, 42%, of all 125 children born with PAIVS had identifiable VCACs. Cumulative survival after surgery was 67%, as compared with 73% for all subjects born with PAIVS (n.s.). Survival improved over time, and there was no late death after 3years of age. Median follow-up time was 12years (range 1–28years). At follow-up, 33 subjects were alive (12 female/21 male) with the following repairs: six had a biventricular repair, one had 1.5 ventricle repair, 25 were palliated with a single ventricle repair (final stage in 20), and one had a heart transplantation. Notably after decompression ( n =9 survivors), the disease in the coronary arteries did not progress, although four subjects in this group had large VCACs. In the group of subjects with single ventricle repair and large VCACs, eleven were found to have stenoses in the coronary arteries. The disease in the coronary arteries in this group, however, often progressed with an adverse effect on myocardial perfusion. Four of the survivors had documented myocardial infarctions.

Objective: To describe clinical features, morphology, management and outcome of pulmonary vein stenosis (PVS) in childhood. Design and setting: Retrospective international collaborative study involving 19 paediatric cardiology centres in the UK, Ireland and Sweden. Patients: Cases of PVS presenting between 1 January 1995 and 31 December 2004 were identified. Cases where pulmonary veins connected to a morphological left atrium were included. Functionally univentricular hearts and total anomalous pulmonary venous connection were excluded. All available data and imaging were reviewed. Results: 58 cases were identified. In 22 cases (38%) there was premature delivery. 46 (79%) had associated cardiac lesions; 16 (28%) had undergone previous cardiac surgery before PVS diagnosis. 16 children (28%) had a syndrome or significant extracardiac abnormality. 36 presented with unilateral disease of which 86% was on the left. Where there was adequate sequential imaging, disease progression was shown with discrete stenosis leading to diffusely small pulmonary veins. Collateral vessels often developed. 13 patients had no intervention. Initial intervention was by catheter in 17 and surgery in 28. Overall 3-year survival was 49% (95% CI 35% to 63%) with patients undergoing initial surgical intervention having greater freedom from death or re-intervention (hazard ratio 0.44, 95% CI 0.2 to 0.99, p = 0.023). Conclusions: PVS is a complex disease of uncertain cause and frequently associated with prematurity. Early intervention may be indicated to deter irreversible secondary changes.

Early cardiac complications after autologous bone marrow transplantation (ABMT) were recorded for 49 children with haematological malignancies. There was no procedure-related mortality and only two cases of early post-transplant cardiac complications of clinical relevance, both of which were reversible. For 35 long-time survivors (median follow-up 7 y) serial evaluations before and after ABMT included ECG, chest radiography, echocardiography and equilibrium radionuclide ventriculography (RVG). One patient had frequent supraventricular ectopic beats after ABMT, a finding not previously noted. The mean left ventricular diastolic diameter (LVDD) was 104% of expected before ABMT (95% confidence interval 99-110). During the first year post-transplant LVDD was about 110% of expected, but thereafter normalization occurred. The mean shortening fraction before ABMT was 31% (CI 29-34), compared with the mean value of 34% for healthy children in our laboratory, and it ranged between 29% and 33% during the follow-up period. Mean left ventricular ejection fraction determined by RVG was 65% (CI 61-69) and mean right ventricular ejection fraction was 46% (CI 43-49) before ABMT, and they did not change during follow-up. It is encouraging that these heavily pre-treated children could be autografted without serious cardiac complications or deterioration in myocardial performance in a 5-10-y prospect, but longer follow-up is needed for a final evaluation.

Aim: To determine what proportion of newborns with critical heart defects are discharged undiagnosed from the maternity ward. Methods: Data were retrieved retrospectively for infants born in our referral area from 1993 to 2001 and undergoing surgical or catheter-based intervention before 2 mo of age because of critical heart defects. Results: 259 full-term infants had critical heart defects. Duct-dependent systemic circulation was present in 129, duct-dependent pulmonary circulation in 106, and 24 infants were not duct dependent but critically ill. In 51 infants (20%) the heart defect was not suspected before discharge from the maternity ward. Such late detection occurred more often in infants with duct-dependent systemic circulation (30%) and in children with defects that were not duct dependent (38%) than in children with duct-dependent pulmonary circulation (4%) (p

Background We hypothesized that blood cardioplegia preserves myocardial metabolism and function more effectively than St Thomas' crystalloid cardioplegia in infant cardiac surgery. Methods Thirty infants with atrioventricular septal defects were randomly allocated to either blood or crystalloid intermittent cold (4°C) cardioplegia. Arterial and coronary sinus blood was analyzed for lactate and oxygen. Cardiac output (thermodilution) and left ventricular function (echocardiography) were evaluated. Results The lactate concentration in coronary sinus blood early after bypass was significantly higher after crystalloid cardioplegia than after blood cardioplegia (2.1 ± 0.3 vs 1.3 ± 0.1 mmol/L, p = 0.006), with a significant myocardial release of lactate after crystalloid but not after blood cardioplegia. Oxygen extraction (arterial–coronary sinus O 2 content) was higher early after crystalloid cardioplegia (3.02 ± 0.13 vs 2.35 ± 0.22 mmol/L, p = 0.01), possibly reflecting a difference in oxygen debt. The cardiac index was higher after blood cardioplegia (4.9 ± 0.3 vs 4.0 ± 0.3 L/min −1/ m −2 , p = 0.04) and echocardiographic grading of left ventricular function was better (4.1 ± 0.17 vs 3.5 ± 0.22 arbitrary units, p = 0.046). Conclusions This study indicates that blood cardioplegia preserves myocardial metabolism and function more effectively than crystalloid cardioplegia in infant cardiac surgery. The clinical significance of this finding is uncertain, but the more than 20% increase in cardiac index in the critical phase during weaning from bypass may be advantageous.

Objective: To measure quality of life in children with pulmonary atresia and intact ventricular septum, and make comparisons with a healthy group of children from the general Swedish population. An additional aim was to compare quality of life in two subgroups, children undergoing biventricular and univentricular repair, the latter by establishment of the Fontan circulation. Methods: Quality of life was measured using a mailed questionnaire, taking into consideration the three spheres of personal, interpersonal, and external living conditions. In all, 52 children received the questionnaire, and the answers were compared with those of a random sample of 1856 healthy Swedish children. Results: We received responses from 42 children and parents. The overall differences between groups were small, and no difference was found when comparing the children undergoing univentricular as opposed to biventricular surgical repair. In terms of personal quality of life, we found a higher level of psychosomatic complaints, and lower satisfaction with their own and parent–child activities, in those with the congenital anomaly than in their normal peers. In terms of the interpersonal sphere, more time was available for those with malformed hearts, but the experience of support from the relatives was significantly lower. Conclusions: Overall, quality of life was equal between children with pulmonary atresia and intact ventricular septum and the children in the reference group. The personal psychological conditions were less favourable for children with pulmonary atresia and intact ventricular septum. The perceived lack of support from relatives was also higher in these families. No difference was found when comparing the children undergoing univentricular and biventricular surgical repair.

To study the outcome of all children born with pulmonary atresia and intact ventricular septum in Sweden between 1980 and 1999.Retrospective study of medical records with review of the initial, preoperative angiocardiographic and echocardiographic examinations.A total of 84 children were born with pulmonary atresia and intact ventricular septum, giving an incidence of 4.2 per 100,000 live births. In all, 77 were operated on with a 1-year survival rate of 75%. Thirty-six children had ventriculocoronary communications, with a 1-year survival rate of 50%. At the end of the study period, 52 children were alive, 32 with biventricular repair, and 19 with univentricular repair. Follow-up time was 14 days-20 years (median, 6 years). Statistical analysis of incremental risk factors for death showed statistical significance for low birth weight, male sex, muscular pulmonary atresia, and having a systemic-to-pulmonary shunt as the sole initial intervention.Complete national data of all patients born with pulmonary atresia and intact ventricular septum during 1980-1999 in Sweden revealed a total mortality in accordance with previous reports for results for surgery. Further improvements demand a thorough preoperative investigation of the cardiac anatomy, particularly of the ventriculocoronary communications, to enable right ventricular decompression whenever possible.

OBJECTIVE—To study short and long term results after surgical closure of isolated ventricular septal defects (VSDs) from 1976 to 1996, especially in relation to changes in preoperative evaluation during this period. DESIGN—Retrospective study. SETTING—Tertiary referral centre for paediatric cardiac care. PATIENTS—All children under 18 years of age who had corrective surgery for VSD between 1976 and 1996. MAIN OUTCOME MEASURES—Preoperative evaluation, indications for surgery, diagnostic errors, and early and late results. RESULTS—A significant decrease in the use of invasive preoperative studies in favour of non-invasive methods was found; from 1976 to 1990, a total of 109/110 patients had preoperative invasive study as compared to 43/167 from 1991 to 1996 (p

OBJECTIVES—To describe the evaluation, decision making, and care of children with a complete atrioventricular septal defect (CAVSD). STUDY DESIGN—Retrospective study of 136 consecutive cases from 1970 to 1996. RESULTS—A total of 115 (85%) children had Down's syndrome. Denial of surgery without obvious medical reasons was more common in the early years, as was parental refusal of offered surgery and institutional care of the children. Improved results in later years encouraged surgical treatment for all these patients, but more liberal attitudes towards patients with Down's syndrome preceded the improved results. The use of echocardiography as a screening method for all newborns with Down's syndrome made it possible to plan for correction within the 1st months of life. CONCLUSIONS—Changing attitudes in society and widespread use of echocardiography have significantly improved the management of children with a CAVSD and Down's syndrome.

UNLABELLED Respiratory syncytial virus (RSV) infection is the leading cause of infection of the lower respiratory tract in infants, and is also the leading cause of hospitalization during the first year of life. In this issue of Acta Paediatrica, Meberg and Bru report on the incidence and estimated costs of hospitalizations for RSV infections in children with congenital heart disease from a specified region in Norway. CONCLUSION Incidence figures of hospitalization due to RSV infection in this high-risk group of infants were similar to the results from several other reports. The cost-benefit analysis of prophylaxis against RSV infection in infants with congenital heart disease, using the monoclonal antibody palivizumab, should also take into account potential savings attributable to such prophylaxis.

The procedure of total cavopulmonary connexion includes trans-section and closure of the pulmonary trunk, the systemic venous blood flow being directed to the pulmonary vascular bed by an anastomosis of the superior caval vein with the right pulmonary artery. Following this procedure, there remains a small residual pulmonary trunk. The purpose of our study was to obtain information about the fate of this residual trunk and its contained pulmonary valve. Using transthoracic echocardiography, we examined 29 patients (mean age 7 years 4 months) after a total cavopulmonary connexion (mean interval 17 months). We were able to obtain adequate visualization of the pulmonary valve and the pulmonary trunk in 23 of the patients. Pulmonary regurgitation was demonstrated by colour Doppler echocardiography in 13 pts (57%). Formation of thrombus in the residual pulmonary trunk was detected in one patient (4%) 6 months after the operation. One cerebrovascular embolic event occurred in a patient who had pulmonary regurgitation without formation of thrombus in the residual trunk 3 months after the total cavopulmonary connexion. Our findings show that pulmonary regurgitation is frequently present in patients after total cavopulmonary connexion. Although formation of thrombus in the residual pulmonary trunk seems to be less common, we recommend surgical closure of the pulmonary valve during this procedure so as to exclude the residual pulmonary trunk as a possible source of systemic emboli.

Aim: This is the first study to evaluate compliance with the 2003 Swedish national guidelines for prophylactic treatment of respiratory syncytial virus (RSV) in children with congenital heart disease (CHD). We estimated the relative risk (RR) of children with CHD being hospitalised with a RSV infection, studied the extent to which RSV prophylactic treatment with palivizumab corresponded to the guidelines and determined the morbidity of children with CHD who developed RSV infection despite prophylaxis. Methods: This national observational study comprised prospectively registered data on 219 children with CHD treated with palivizumab, medical records on RSV cases and information on hospitalisation rates of children with CHD and RSV infection. Results: The calculated RR of children with CHD being hospitalised with RSV infection was 2.06 (950/0 Cl 1.6-2.6; p < 0.0001) compared with children without CHD. Approximately half of the patients (49%) born before the RSV season and 25% born during the RSV season did not start treatment as recommended by the guidelines. Conclusion: Having CHD increased the rate and estimated RR of children being hospitalised with RSV infection. The guidelines were not followed for about half of the children born before a RSV season and a quarter of the children born during a RSV season and need updating.

Previous studies show that growth hormone (GH) treatment increases cardiac dimensions in short children with GH deficiency (GHD) and has diverse cardiac effects in children with idiopathic short stature (ISS). This study was performed to assess the effect of GH on the cardiovascular system in short children with a broad range of GH secretion and GH sensitivity/responsiveness.In this prospective, multicentre study, short prepubertal children diagnosed with isolated GHD (89) or ISS (38) were followed during 2 years of GH treatment. They were randomized to receive either a standard (43 μg/kg/day) or an individualized GH dose (range 17-100 μg/kg/day) based on GH responsiveness estimated by a prediction model and distance to target height. Echocardiography, blood pressure and electrocardiography were performed at baseline, 3, 12 and 24 months.Left ventricular mass (LVM) indexed to body surface area increased significantly during 2 years of GH treatment in both GHD and ISS irrespective of randomized dose. This change was already apparent at 3 months, when standard deviation scores (SDS) of wall thickness and diameter were increased. At 24 months, left ventricular diameter SDS remained increased, whereas myocardial thickness SDS returned to baseline values. There was no impairment of systolic or diastolic function. There was no correlation with treatment dose and LVM SDS at 24 months.Irrespective of GH status, there was a rapid increase in LVM during GH treatment in short children. At 3 months, wall thickness and diameter were increased, whereas only diameter remained increased at 24 months.

Objective: Pulmonary venous obstruction (PVO) is an important cause of late mortality in total anomalous pulmonary venous connection (TAPVC). We aimed to describe current practices for the management of postoperative PVO and the efficacy of the different interventional procedures. Methods: We conducted a retrospective international collaborative population-based study involving 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. Patients with TAPVC born between January 1, 1998, and December 31, 2004, were identified. Patients with functionally univentricular circulation or atrial isomerism were excluded. All available data and images were reviewed. Results: Of 406 patients undergoing repair of TAPVC, 71 (17.5%) had postoperative PVO. The diagnosis was made within 6 months of surgery in 59 (83%) of the 71 patients. In 12, serial imaging documented change in appearance of the pulmonary veins. Good-sized pulmonary veins can progress to diffusely small veins and rarely atresia. Patients presenting after 6 months had less severe disease; all are alive at most recent follow-up. Fifty-six (13.8%) of 406 patients underwent intervention for postoperative PVO: 44 had surgical treatment and 12 had an initial catheter intervention. One half underwent 1 or more reinterventions. Three-year survival for patients with postoperative PVO was 58.7%(95% confidence intervals, 46.2%-69.2%) with a trend that those having a surgical strategy did better (P = .083). Risk factors for death included earlier presentation after TAPVC repair, diffusely small pulmonary veins at presentation of postoperative PVO, and an increased number of lung segments affected by obstruction. Conclusions: Postoperative PVO tends to appear in the first 6 months after TAPVC repair and can be progressive. Early intervention for PVO may be indicated before irreversible secondary changes occur. (J Thorac Cardiovasc Surg 2013;145:1255-62)

To investigate physical activity, sports participation and aerobic fitness in children who have undergone surgery for congenital heart defects.Children who had undergone surgery for congenital heart defects and healthy controls in the Gothenburg area were invited to participate in the study. All participants were aged 9-11 or 14-16 years. The activity monitor ActiReg was used to assess physical activity. Participants were interviewed about their participation in sports and performed a maximal exercise test on a bicycle with measured oxygen uptake.A total of 32 and 25 patients, and 61 and 45 controls, in the two age-groups were included, respectively. The patients had a wide range of severity of congenital heart defects. The physical activity level was similar in the patients and the controls. The rate of sports participation was high for both patients and controls; 80-94% of all participants took part in sports at least once a week. The majority of the patients were considered to have at least a moderate level of aerobic fitness.Although children who have undergone surgery for congenital heart defects have a similar level of physical activity compared with that of healthy children, some of them may require support to participate in exercise and vigorous physical activity.

The objective of this study was to assess exercise performance in subjects born in Sweden between 1980 and 1995 and undergoing surgery for pulmonary atresia and intact ventricular septum and to identify determinants of exercise performance. Twenty-seven subjects, 16 with biventricular repair and 11 with univentricular palliation, and 28 age- and sex-matched controls completed cardiopulmonary exercise and lung function testing. Peak oxygen uptake was determined using a symptom-limited ramp bicycle exercise protocol. Regression analysis was performed to identify predictors of peak oxygen uptake (V'O(2)), The index group had lower peak V'O(2) (1.4 [median 0.8; range 2.5] l/min) than controls (1.9 [0.7; 3.1]; p < 0.05). Subjects without ventriculocoronary arterial communications (VCAC), corrected to biventricular circulation, had higher peak V'O(2), than the remaining index subjects. Decreased total lung capacity, low minute ventilation, and high physiologic dead space measured at peak exercise were all independent determinants of low peak V'O(2) Exercise capacity is generally decreased in subjects with pulmonary atresia and intact ventricular septum, although there are marked interindividual differences. Good exercise capacity was found in subjects without VCAC who had undergone biventricular repair. Decreased lung function was an unfavourable predictor of exercise capacity.

GH may exert direct growth-promoting and metabolic actions on target tissues, but most of its effects are mediated by circulating (endocrine) or local (auto-/paracrine) IGF-I. The GH/IGF-I system has an important role in cardiac development and in maintaining the structure and function of the heart. A subgroup of children with pronounced heart defects will eventually need transplants, owing to congestive heart failure. Since the symptoms are often severe and may progress while waiting for surgery, it is necessary to develop supportive medical treatment. GH has been proposed as a therapeutic agent in adults with heart failure, but to date studies are lacking on children and more information is necessary. We have examined the expression of IGF-I mRNA and GH-receptor (GH-R) mRNA in children undergoing surgery for congenital heart disease. Eighteen children scheduled for open-heart surgery were included in the study. Right auricular biopsies were taken at the time of venous catheterization preceding cardiac bypass. The specimens were analysed using realtime PCR. We were able to show expression of both IGF-I mRNA and GH-R mRNA in the pediatric heart. The relative expressions were intercorrelated (r=0.75, p

We describe the results of myocardial perfusion scintigraphy performed 4 to 15 years after surgery in 12 patients with pulmonary atresia and intact ventricular septum. We used single photon emission computed tomography after injection of technetium Tc-99m tetrofosmin at submaximal exercise test. The patients, 7 girls and 5 boys, with a mean age of 11 years, and a range from 6 to 19 years, had either undergone biventricular repair, in 5 cases, or univentricular palliation in the remaining 7. This second group included 4 patients with ventriculo-coronary arterial communications. Of the children, 3 with biventricular repair and 6 with univentricular palliation had perfusion defects. Children with biventricular repair had perfusion defects in the ventricular septum, while those having univentricular palliation also had defects located to the left ventricular free wall. All children with ventriculo-coronary arterial communications had perfusion defects both in the ventricular septum and in the left ventricular free wall.

Aim: The myocardial uptake of substrates in children has only been investigated on a small scale. The purpose of this study was to define myocardial substrate uptake in relation to the arterial supply of substrates, age, growth and oxygen saturation. Methods: Thirty patients with congenital heart disease, aged 3 months to 16 years, were studied during cardiac catheterization. Arterial and coronary sinus blood was analyzed for the major fuel metabolites and amino acids. Results: The uptake of all major substrates correlated significantly with the arterial supply: free fatty acids (r = 0.52, p = 0.004), β-hydroxybutyrate (r = 0.74, p < 0.0001), lactate (r = 0.70, p < 0.0001) and glucose (r = 0.48, p = 0.01). Free fatty acids were the dominant substrate, irrespective of age, growth and saturation. With age, there was an increase in the uptake of lactate (r = 0.61, p = 0.0004) and a decrease in the uptake of β-hydroxybutyrate (r =–0.41, p = 0.02). In multivariate analyses, these changes were explained by the arterial supply of the substrates, while age per se did not contribute significantly. Conclusion: The uptake of myocardial metabolites correlated with the arterial supply. Free fatty acids were the dominant substrate at all ages. The uptake of lactate and β-hydroxybutyrate, although varying with age, was also determined by the arterial supply.

To evaluate the feasibility of detecting duct-dependent congenital heart disease before hospital discharge by using pulse oximetry.Case-control study.A supra-regional referral centre for paediatric cardiac surgery in Sweden.200 normal term newborns with echocardiographically normal hearts (median age 1.0 d) and 66 infants with critical congenital heart disease (CCHD; median age 3 d).Pulse oximetry was performed in the right hand and one foot using a new-generation pulse oximeter (NGoxi) and a conventional-technology oximeter (CToxi).With the NGoxi, normal newborns showed a median postductal saturation of 99% (range 94-100%); intra-observer variability showed a mean difference of 0% (SD 1.3%), and inter-observer variability was 0% (SD 1.5%). The CToxi recorded a significantly greater proportion of postductal values below 95% (41% vs 1%) in the normal newborns compared with NGoxi (p0.0001). The CCHD group showed a median postductal saturation of 90% (45-99%) with the NGoxi. Analysis of distributions suggested a screening cut-off of95%; however, this still gave 7/66 false-negative patients, all with aortic arch obstruction. Best sensitivity was obtained by adding one further criterion: saturation of95% in both hand and foot or a difference of+/-3% between hand and foot. These combined criteria gave a sensitivity of 98.5%, specificity of 96.0%, positive predictive value of 89.0% and negative predictive value of 99.5%.Systematic screening for CCHD with high accuracy requires a new-generation oximeter, and comparison of saturation values from the right hand and one foot substantially improves the detection of CCHD.

In Sweden, which has a population of 8.9 million people, pediatric heart surgery was previously performed in four cities. After a long, difficult process, centralization of pediatric heart surgery to two centers was achieved in 1993. The overall 30-day mortality for open-heart surgery on infants and children of 9.5% before the centralization (1988-1991) was reduced to 1.9% in 1995-1997. A causal relationship between the mortality rates before and after the centralization is impossible to prove. Heart surgery was concentrated to the two centers with the lowest surgical mortality, and the reduction in surgical mortality was observed over a short period of time which makes it likely that the centralization of the surgical activity promoted the improved results. During the later time period the amount of more complex surgery was clearly increased compared to that performed previously.

A study was carried out to assess significant short-term postoperative complications in children undergoing valved homograft surgery for congenital heart defects. Clinical and laboratory parameters of 50 patients undergoing this type of surgery were compared with those of 50 randomly selected but age-matched controls in whom open-heart surgery was performed without a homograft. Extracorporeal circulation, aortic clamping and intensive care times and the duration of febrile response were significantly longer in the study patients than in the controls (each p < 0.0001). In laboratory data the only significant difference between the two groups was in the C-reactive protein response, which was high in the study group (p < 0.0004) and did not correlate with extracorporeal circulation time or with positive blood cultures. The prolonged febrile and acute-phase reactant response following valved homograft surgery is an inflammatory reaction to the foreign antigen.

A case of absent right aortic cusp causing serious aortic incompetence in a neonate is reported. A "bicuspidalization" repair broke down within 24 hours, but reoperation with annular enlargement and insertion of a 19-mm prosthetic valve resulted in complete recovery. This rare but life-threatening anomaly of the aortic valve can be recognized with Doppler echocardiography. Valve replacement in conjunction with aortic annular enlargement should be performed as primary repair.

Fourty-six adult patients operated on as chidren with resection and end to end anastomosis because of coarctation of the aorta were studied. The age at operation was 7 to 13 years (mean age 10 years) and at follow-up 18 to 28 years (mean age 21 years). Arm and thigh cuff pressure was measured at rest, during and immediately after submaximal exercise. The systolic gradients were also estimated using continuous wave Doppler. Systolic blood pressure at rest was 150 mmHg or more in 12 patients (26%). The cuff pressure at rest correlated well with arm-leg gradients at rest and at work, and also with the Doppler gradients at rest. Doppler gradients slightly underestimated invasively measured gradients. The results imply that postoperative hypertension was explained by residual mechanical obstruction.

A girl with severe neonatal hypocalcaemia, thymic hypoplasia, congenital heart disease and mental retardation in combination with a partial monosomy of chromosome 22, del(22)(pter-q11.3), is reported. Nine other patients with an association between partial monosomy 22 and a DiGeorge syndrome have been reported earlier, and this combination probably constitutes a deletion syndrome similar to the Prader-Willi and the aniridia-Wilms' tumour syndromes. However, the deletion of chromosome 22 is mostly due to a translocation, with trisomy for another chromosomal segment. Such a mechanism may explain the different clinical features seen in patients with partial monosomy 22. In the present case there was an unbalanced translocation with a probable trisomy of the short arm of chromosome 20 combined with the partial monosomy 22. Cytogenetic investigation with high resolution banding techniques is indicated in patients with thymic aplasia and suspected DiGeorge syndrome.

Purpose Numerous studies have investigated causes of warfarin dose variability in adults, whereas studies in children are limited both in numbers and size. Mechanism-based population modelling provides an opportunity to condense and propagate prior knowledge from one population to another. The main objectives with this study were to evaluate the predictive performance of a theoretically bridged adult warfarin model in children, and to compare accuracy in dose prediction relative to published warfarin algorithms for children. Method An adult population pharmacokinetic/pharmacodynamic (PK/PD) model for warfarin, with CYP2C9 and VKORC1 genotype, age and target international normalized ratio (INR) as dose predictors, was bridged to children using allometric scaling methods. Its predictive properties were evaluated in an external data set of children 0–18 years old, including comparison of dose prediction accuracy with three pharmacogenetics-based algorithms for children. Results Overall, the bridged model predicted INR response well in 64 warfarin-treated Swedish children (median age 4.3 years), but with a tendency to overpredict INR in children ≤2 years old. The bridged model predicted 20 of 49 children (41 %) within ± 20 % of actual maintenance dose (median age 7.2 years). In comparison, the published dosing algorithms predicted 33–41 % of the children within ±20 % of actual dose. Dose optimization with the bridged model based on up to three individual INR observations increased the proportion within ±20 % of actual dose to 70 %. Conclusion A mechanism-based population model developed on adult data provides a promising first step towards more individualized warfarin therapy in children. Electronic supplementary material The online version of this article (doi:10.1007/s00228-012-1466-4) contains supplementary material, which is available to authorized users.

Thirteen children with Friedreich's ataxia were reviewed. The clinical presentation and evolution of the disease was compared to that observed in large series—based mainly on adult patients—and the few studies in children. The mean age of onset (5.3 ± 2.7 years) was lower than that reported in the former studies. Progressive unremitting ataxia of all four limbs was the earliest and most consistent finding, whereas dysarthria and loss of joint or vibration sense occurred with less frequency than that reported in adult series. The tendon jerks were absent or reduced in the lower limbs in almost all children. The universal absence of lower limb reflexes was shown to be too rigid to be obligatory for the diagnosis of early cases of Friedreich's ataxia. Electrophysiologic investigations revealed typical findings, ie, normal or low-normal motor conduction velocities and absent sensory responses. Electromyography showed more features of denervation in the lower limbs than in the upper limbs. Cardiac symptoms and signs were minimal, whereas electrocardiographic abnormalities occurred in 92% of patients, presenting mostly as significant T-wave changes. Concentric symmetric thickening of both the interventricular and left ventricular posterior walls was the major echocardiographic finding. ( J Child Neurol 1990;5:321-326).