Academic achievement and satisfaction in adolescents with CHD.

OBJECTIVES: To evaluate academic achievement and satisfaction in adolescents with CHD. Study design Questionnaires were sent to all adolescents, aged between 17 and 20 years with CHD, currently treated at our hospital (n=326) in order to assess the patients' education and satisfaction with their academic career. Results were compared with the official community statistics. RESULTS: A total of 207 patients completed the questionnaires (participation rate 63.5%), 113 boys and 94 girls; 50% had completed mandatory school at the highest, 37.3% at the middle, and 12.7% at the lowest educational level. The distribution in the general population was comparable: 57.6, 32.5, and 9.9%, respectively (p=0.8). Adolescents with severe CHD were less likely to attain a higher educational level than those with moderate or mild CHD (p=0.03 for school grades 7-9). None of the other examined medical or socio-demographic factors, such as socio-economic status, foreign language, severity of CHD, cyanosis, and open heart surgery, were found to be associated with lower educational attainment. After the mandatory 9 years of schooling, 21.4% (n=44) of the patients with CHD compared with 16.7% in the general population attended higher school levels heading towards university education (p=0.7). From the 165 patients who provided information on career satisfaction, 79% regarded their job or school situation as being their desired one without a difference for those with severe CHD. CONCLUSION: School education in Swiss adolescents with CHD is very similar to the normal population. In addition, the majority of adolescents are satisfied with their educational career. This fact may be due to the good educational support provided during schooling.

Pharmacological and non-pharmacological therapy for arrhythmias in the pediatric population: EHRA and AEPC-Arrhythmia Working Group joint consensus statement.

In children with structurally normal hearts, the mechanisms of arrhythmias are usually the same as in the adult patient. Some arrhythmias are particularly associated with young age and very rarely seen in adult patients. Arrhythmias in structural heart disease may be associated either with the underlying abnormality or result from surgical intervention. Chronic haemodynamic stress of congenital heart disease (CHD) might create an electrophysiological and anatomic substrate highly favourable for re-entrant arrhythmias. As a general rule, prescription of antiarrhythmic drugs requires a clear diagnosis with electrocardiographic documentation of a given arrhythmia. Risk-benefit analysis of drug therapy should be considered when facing an arrhythmia in a child. Prophylactic antiarrhythmic drug therapy is given only to protect the child from recurrent supraventricular tachycardia during this time span until the disease will eventually cease spontaneously. In the last decades, radiofrequency catheter ablation is progressively used as curative therapy for tachyarrhythmias in children and patients with or without CHD. Even in young children, procedures can be performed with high success rates and low complication rates as shown by several retrospective and prospective paediatric multi-centre studies. Three-dimensional mapping and non-fluoroscopic navigation techniques and enhanced catheter technology have further improved safety and efficacy even in CHD patients with complex arrhythmias. During last decades, cardiac devices (pacemakers and implantable cardiac defibrillator) have developed rapidly. The pacing generator size has diminished and the pacing leads have become progressively thinner. These developments have made application of cardiac pacing in children easier although no dedicated paediatric pacing systems exist.

Management and outcome of Ebstein's anomaly in children.

OBJECTIVES: To assess clinical presentation, treatment, and outcome of children with Ebstein's anomaly. BACKGROUND: Data on long-term outcome of children with Ebstein's anomaly are scarce. METHODS: Retrospective analysis of all children with Ebstein's anomaly treated between February, 1979 and January, 2009 in a single tertiary institution. Primary outcomes included patient survival and need for intervention, either cardiac surgery or catheter intervention. RESULTS: A total of 42 patients were diagnosed with Ebstein's anomaly at a median age of 5 days ranging from 1 day to 11.7 years. Symptoms included cyanosis, heart murmur, and/or dyspnoea. Associated cardiac anomalies occurred in 90% of the patients. Average follow-up was 9.5 plus or minus 7.0 years. The overall mortality rate was 14%. Of the six patients, three died postnatally before treatment. Cardiac surgery and/or catheter-guided interventions were required in 33 patients (79%). Cardiac surgery was performed in 21 (50%) patients at a median age of 9.1 years (range 0.1-16.5 years), including biventricular repair in 13 (62%), one-and-a-half chamber repair in seven (33%), and a staged single-ventricle repair in one. Peri-operative mortality was 4%. Catheter-guided interventions consisted of device closure of an atrial septal defect in three cases and radiofrequency ablation of accessory pathways in nine patients. The estimated 10-year survival was 85.3 plus or minus 5.6%. CONCLUSION: In children, Ebstein's anomaly is usually diagnosed in the first year of age. Even though children with Ebstein's anomaly often require an intervention, their peri-operative mortality is low and long-term survival is good. Symptomatic newborns requiring an intervention may have a worse outcome.

Outcome of biventricular repair in infants with multiple left heart obstructive lesions.

The decision to perform biventricular repair for infants with multiple obstructive or hypoplastic left heart lesions (LHL) and borderline left ventricle (LV) may be controversial. This study sought to assess the mortality and morbidity of patients with LHL after biventricular repair and to determine the growth of the left-sided cardiac structures. Retrospective analysis of 39 consecutive infants with LHL who underwent biventricular repair was performed. The median age at surgery was 7 days (range 1-225 days), and the median follow-up period was 34 months (range 1-177 months). Between diagnosis and the end of the follow-up period, the size of the aortic annulus (z-score -4.1 ± 2.8 vs. -0.1 ± 2.7) and the LV (LV end-diastolic diameter z-score -1.7 ± 2.8 vs. 0.21 ± 1.7) normalized. During the follow-up period, 23 patients required 39 reinterventions (62%) consisting of redo surgery for 21 patients (57%) and catheter-guided reinterventions for 8 patients (22%). At the end of the follow-up period, 25 of 34 patients were doing subjectively well; 10 children (29%) received cardiac medication; 12 (35%) presented with failure to thrive (weight ≤ P3) and 5 (15%) with pulmonary hypertension. The overall mortality rate was 13%. Biventricular repair for patients with multiple LHL results in sufficient growth of the left-sided cardiac structures. Nevertheless, residual or newly developing obstructive lesions and pulmonary hypertension are frequent, causing significant morbidity that requires reintervention.

Realignment of the ventricular septum using partial direct closure of the ventricular septal defect in Tetralogy of Fallot.

OBJECTIVE: The aim is to describe our technique of partial direct closure of the ventricular septal defect (VSD) in Tetralogy of Fallot (TOF), and assess its influence on the realignment and remodeling of the left ventricular outflow tract. METHODS: Between 2004 and 2010, 32 non-consecutive patients with TOF underwent a direct or partial direct closure of VSD. Median age and weight were 5.2 months and 6.7 kg, respectively. An approach through the right atrium was used in 30 patients and through the infundibulum in two patients. The conal septum was mobilized by transecting the hypertrophic trabeculae to facilitate the approximation of the VSD. The membranous part of the VSD was closed (in the later part of the series) with a small xenopericardial patch to avoid tension on the suture line traversing the area of risk to the bundle of His. Follow-up was complete, with a median duration of 46.9 (range 12-75.3) months. RESULTS: The VSD could be closed successfully in all patients. A residual VSD was partly responsible for one early postoperative re-operation. There were no early or late deaths. At follow-up, all patients were in sinus rhythm. Three patients showed a small residual VSD. Thirty patients had none, one showed trivial, and one had mild aortic regurgitation. The left ventricular outflow showed a good realignment of the ventricular septum in all the patients. CONCLUSIONS: Partial direct closure of the VSD corrects the primary defect in TOF, that is, the malalignment of the septum. It results in a straight, wide open left ventricular outflow tract and brings better support to the aortic root.

Plasma B-type natriuretic peptide levels in children with heart disease.

AIM: To determine plasma B-type natriuretic peptide (BNP) levels in children with heart diseases before medical or surgical treatment for monitoring therapeutic efficacy in an observational prospective clinical trial at tertiary care centre. METHODS: In 522 paediatric patients at an age of 6.4 ± 5.2 years (mean ± SD; range: 14 days-18 years) with congenital heart disease (CHD), cardiomyopathies (CMP) or pulmonary arterial hypertension (PAH), plasma BNP levels were evaluated before and under treatment. RESULTS: Most types of heart disease are associated with increased mean plasma BNP levels before treatment, with highest values in children with CMP (BNP 6165 pg/mL in dilated CMP vs. 817 pg/mL in hypertrophic, vs. 1236 pg/mL in restrictive CMP, each p < 0.05). Children with PAH showed a significant decrease in BNP levels under medical treatment (mean BNP 981 pg/mL before vs. 26 pg/mL under treatment, p < 0.05). Children with univentricular CHD undergoing surgical staged palliation showed a significant decrease in BNP levels after bidirectional cavopulmonary anastomosis (BDCP) (BNP 109 pg/mL before vs. 70 pg/mL after BDCP, p < 0.05). CONCLUSION: Plasma BNP levels are elevated in children with heart disease before treatment and are a useful laboratory parameter under treatment during long-term follow-up.

Prevalence and predictors of later feeding disorders in children who underwent neonatal cardiac surgery for congenital heart disease.

AIM: We thought of assessing the prevalence and predictors of feeding disorders in patients with congenital heart defects after neonatal cardiac surgery. METHODS: Retrospective study of 82 consecutive neonates (48 males, 34 females) who underwent surgery for congenital heart defects from 1999 to 2002. Information was taken from patient charts and nursing notes. The presence of a feeding disorder was assessed by a questionnaire sent to the paediatricians when the child was 2 years of age. A feeding disorder was defined as a need for tube feeding, inadequate food intake for age, or failure to thrive. Data were analysed with descriptive statistics and logistic regression. RESULTS: Feeding disorders occurred in 22% of the study population. Reoperation and early feeding disorders were identified as independent risk factors for later feeding disorders (odds ratio 5.8, p 0.01; odds ratio 20.7, p 0.02). There was a trend towards more feeding disorders in patients with neurological abnormalities during the first hospital stay. CONCLUSION: Feeding disorder is a frequent, long-term sequela after neonatal cardiac surgery. Patients with congenital heart defects who undergo multiple cardiac surgeries and those with early feeding disorders are at risk of developing later feeding disorders. Patients with these risk factors need to be selected for preventive strategies.

Safety and efficacy of paediatric outpatient radiofrequency catheter ablations.

AIMS: Radiofrequency catheter ablation (RFA) is a frequently performed procedure as treatment for supraventricular tachycardia in children >4 years of age. The aim of this study was to evaluate the safety and efficacy of the paediatric outpatient RFA procedure. METHODS: Between 2002 and 2008, 271 RFAs were analyzed. Exclusion criteria for outpatient procedures amongst others were distance to home >1h and anticipated complex RFA in congenital heart disease. All patients underwent pre- and post-procedural echocardiography and electrocardiogram. Patient discharge was within 6h after conclusion of RFA. Parental follow-up phone calls the day after the procedure were performed. All patients were seen in outpatient clinics 1 month after RFA. RESULTS: A total of 97/271 (37%) patients aged 13.6 (4.8-18.0) years qualified for outpatient RFA. Accessory pathway ablations (n=50) and atrioventricular node modifications (n=39) were the most common RFAs. RFA was successful in 94/97 (97%) patients. Post-procedural echocardiography and electrocardiogram did not reveal any RFA related complications. Anaesthetic adverse events, predominantly post-interventional nausea and vomiting, were observed in 10 (10%) patients. Hospital discharge within 6h was practicable in all but one due to ongoing nausea. Follow-up phone calls did not reveal further complications. Recurrence of tachycardia after successful RFA was observed in 6 of 94 (6%) patients and prompted re-intervention in 4. CONCLUSIONS: Outpatient RFA is feasible and safe in selected paediatric patients. No RFA related complication was observed. Anaesthetic adverse events were nausea and vomiting due to general anaesthesia. Success rate and recurrence rate of tachycardia were favourable after outpatient RFA.

Risk factor analysis of 170 single-institutional contegra implantations in pulmonary position.

BACKGROUND: The aim of this study is to evaluate risk factors affecting survival of Contegra grafts used in the pulmonary position. METHODS: One hundred seventy Contegra implanted (2001 to 2007) in the pulmonary position for replacement after a prior repair (90), Ross procedure (29), tetralogy of Fallot and variants (22), truncus arteriosus (13), Rastelli procedure (8), and miscellaneous (8) were reviewed. Median age was 107 (0.1 to 894) months. Follow-up was 96% complete with a median duration of 65 (7 to 98) months. RESULTS: There were 7 early and 6 late deaths (none Contegra related) with a survival of 92%±2% at 98 months. Thirty-four Contegra were replaced at a median duration of 43 (7 to 82) months. Eight of 28 balloon-dilated Contegra remain palliated at 49 (23 to 73) months. Multivariate analysis showed Contegra less than 16 mm (p<0.001; HR [hazard ratio] 0.07), high pulmonary pressure (p<0.001; HR 4), and prior operation era (p=0.006; HR 0.3) as independent risk factors for Contegra replacement. The freedom from replacement for Contegra less than 16 mm and 16 mm or greater were 48%±8% and 98%±2%; for presence and absence of high pulmonary pressure were 52%±11% and 88%±3% and for era 1 and 2 were 77%±5% and 88%±4% at 60 months, respectively. Twenty-three of 123 surviving Contegra have a mean Doppler gradient greater than 20 mm Hg. Contegra less than 16 mm, smaller age group, obstructive arborization, and era 1 were significantly associated with higher gradients. While 18 (15%) surviving Contegra have moderate or more regurgitation, 88 (72%) have no significant gradient or regurgitation. CONCLUSIONS: Contegra grafts integrate well into the body. Larger Contegra show predictable function with a survival approaching homografts over medium term. Better case selection as one ascends the learning curve tends to improve survival. With easy availability and predictable quality, Contegra grafts continue to be a promising complement to homografts.

Sudden cardiac death: clinical evaluation of paediatric family members.

AIMS: To evaluate paediatric relatives of first- and non-first-degree family victims with a history of premature sudden cardiac death (SCD) or aborted cardiac arrest (ACA). METHODS AND RESULTS: Thirty-six consecutive referred families after SCD (n = 29) or ACA (n = 7) of a family member were analysed. Referral was either due to an inherited heart disease identified after autopsy, post-event, or family evaluation (n = 20 families) or due to sudden unexplained death (SUD, n = 16 families). In 3 of 16 (19%) SUD families, an inherited heart disease was diagnosed by evaluation of the paediatric relatives. In 5 of 25 (20%) referred paediatric relatives of SUD families, an inherited heart disease was identified, mainly sinus node dysfunction (n = 3). A total of 13 of 33 (39%) referred paediatric relatives of families with known inherited heart disease were affected, mainly with cardiomyopathy (n = 5) and primary electrical disease (n = 7). Prevention of SCD was initiated in 16 of the affected children by implantation of an antibradycardia device (n = 3), an implantable cardioverter defibrillator (ICD, n = 6), and/or antiarrhythmic medication (n = 8). Appropriate and successful ICD discharges occurred in four. CONCLUSION: A stepwise, comprehensive clinical investigation of SCD or ACA families identifies a substantial number of paediatric relatives at risk of SCD. This allows for targeted prevention by effective treatments and evaluation of further relatives.

Do predictors exist for a successful withdrawal of preoperative prostaglandin E(1) from neonates with d-transposition of the great arteries and intact ventricular septum?

Prostaglandin E(1) (PGE(1)) is given to neonates with d-transposition of the great arteries (d-TGA) to reduce cyanosis by reopening and maintaining the patency of the ductus arteriosus. To avoid side effects, this medication can be stopped for hemodynamically stable patients after balloon atrial septostomy (BAS). A consecutive series of neonates with d-TGA and an intact ventricular septum (IVS) presenting from 2000 through 2005 was analyzed retrospectively to search for side effects of PGE(1) and to identify predictors for a safe preoperative withdrawal. The medication was stopped for hemodynamically stable patients with transcutaneous oxygen saturations higher than 80% after BAS and reinitiated for patients with an oxygen saturation lower than 65%. Patients successfully weaned were compared with those who had failed weaning in terms of atrial septal defect (ASD) size, ductus arteriosus size, and the transcutaneous oxygen saturation. Prostaglandin E(1) was initiated for all 43 neonates with d-TGA. The median maintenance dose of PGE(1) was 0.00625 µg/kg/min (range, 0.00313-0.050 µg/kg/min) for a median duration of 6 days (range, 1-12 days). For 16 patients, PGE(1) was preoperatively withdrawn but then had to be reinitiated for 7 of the 16 patients. No predictors for a successful weaning of PGE(1) were found based on ASD size, ductus arteriosus size, or oxygen saturation. The adverse effects of PGE(1) were apnea in 10 patients and fever in 19 patients. Neither seizures nor necrotizing enterocolitis was documented. Prostaglandin E(1) was successfully withdrawn for a minority of hemodynamically stable patients with d-TGA. No predictors for a successful weaning could be identified. Because apnea and fever are common side effects, withdrawal of PGE(1) after BAS may improve patient safety and comfort. In this patient group, if PGE(1) withdrawal was not well tolerated, it could be safely reinitiated. There were no serious side effects of PGE(1.).

Angiotensin-converting enzyme inhibitors in pediatric patients with mitral valve regurgitation-case-control study and review of the literature.

OBJECTIVES: To investigate the effectiveness of chronic administration of angiotensin-converting enzyme inhibitors in pediatric patients with mitral valve regurgitation. DESIGN, SETTING, PATIENTS, INTERVENTIONS: This was a case-control study of all echocardiograms of patients with moderate-to-severe mitral valve regurgitation, who underwent chronic administration of angiotensin-converting enzyme inhibitors between January 1990 and December 2006 at a single center. OUTCOME MEASURES: Echocardiographic parameters (left ventricular end-diastolic diameter, left ventricular posterior wall diameter, interventricular septum diameter, left atrium to aortic root diameter ratio, grade of mitral valve regurgitation, shortening fraction) were analyzed before and during therapy with angiotensin-converting enzyme inhibitors in 12 patients and compared with 12 patients without medications after one month and one year. RESULTS: Twenty-four consecutive pediatric patients (median age of 7 years with a range 1 month-16 years) with moderate-to-severe mitral valve regurgitation were included. Data are given as standard deviation scores (z-scores) derived from body-surface-adjusted normal values. During angiotensin-converting enzyme inhibition left ventricular end-diastolic diameter decreased from mean z-score 2.04 to 1.66 (after 1 month) and to 1.73 (after 1 year), while left ventricular posterior wall diameter decreased from 0.25 to 0.12 (after 1 year), respectively. Shortening fraction, interventricular septum diameter, grade of mitral valve regurgitation, and left atrium to aortic root diameter ratio remained stable. CONCLUSIONS: In this case-control study of patients with moderate-to-severe mitral valve regurgitation effectiveness of angiotensin-converting enzyme inhibition on left ventricular dimensions and function after 1 month and 1 year is limited. Reviewing the literature, the lack of long-term follow-up studies with large patient cohorts and controversial study-results in adults require a prospective long-term multicenter follow-up study in pediatric patients.

Determinants of body weight gain and association with neurodevelopmental outcome in infants operated for congenital heart disease.

The aim of this prospective study was to examine the determinants of growth failure and the association with neurodevelopmental outcome in infants undergoing open-heart surgery. In 107 infants undergoing open-heart surgery for congenital heart disease (CHD), we evaluated weight at birth, at surgery, and at one year of age (expressed as z-scores). Neurodevelopmental status was assessed before surgery and at one year of age. Median age at surgery was 3.9 months (range: 0.1-10.2). Mean [+/-standard deviation (S.D.)] weight z-score at birth was -0.27 (+/-1.45), before surgery -1.34 (+/-1.45) (P<0.001 vs. birth weight), and at one year -0.86 (+/-1.35), (P<0.001 vs. weight at surgery). Poor preoperative weight (<10th percentile) was associated with genetic disorders [odds ratio (OR) 5.9, P<0.001], preoperative neurological abnormalities (OR 3.41, P<0.05), and older age at surgery (OR 1.01, P<0.05). Weight <10th percentile at one year was associated with the same factors as poor preoperative weight, however, also with risk adjustment for congenital heart surgery-1 (RACHS) score >3 (OR 3.22, P<0.05). Neurodevelopmental outcome at one year was not determined by growth failure. In conclusion, impaired body weight gain before surgery is followed by a catch-up growth after surgery. However, there is no relationship to neurodevelopmental outcome. Genetic comorbidity is the most significant factor for poor weight gain.

Feasibility of perfusion cardiovascular magnetic resonance in paediatric patients.

AIMS: As coronary artery disease may also occur during childhood in some specific conditions, we sought to assess the feasibility and accuracy of perfusion cardiovascular magnetic resonance (CMR) in paediatric patients. METHODS AND RESULTS: First-pass perfusion CMR studies were performed under pharmacological stress with adenosine and by using a hybrid echo-planar pulse sequence with slice-selective saturation recovery preparation. Fifty-six perfusion CMR examinations were performed in 47 patients. The median age was 12 years (1 month-18 years), and weight 42.8 kg (2.6-82 kg). General anaesthesia was required in 18 patients. Mean examination time was 67 +/- 19 min. Diagnostic image quality was obtained in 54/56 examinations. In 23 cases the acquisition parameters were adapted to patient's size. Perfusion CMR was abnormal in 16 examinations. The perfusion defects affected the territory of the left anterior descending coronary artery in 11, of the right coronary artery in 3, and of the circumflex coronary artery in 2 cases. Compared to coronary angiography, perfusion CMR showed a sensitivity of 87% (CI 52-97%) and a specificity of 95% (CI 79-99%). CONCLUSION: In children, perfusion CMR is feasible and accurate. In very young children (less than 1 year old), diagnostic image quality may be limited.

Self-monitoring of oral anticoagulation therapy in children.

This study aimed to investigate the accuracy of home International Normalized Ratio (INR) self-monitoring in pediatric patients on long-term oral anticoagulation therapy. Statistical and clinical agreement of INR values from capillary whole blood samples measured by 2 different portable prothrombin time monitors (CoaguChek S and XS) and venous blood samples measured by a laboratory coagulation analyzer were evaluated using the Bland-Altman analysis. Eighty-three INR comparisons (56 using the CoaguChek S and 27 using the CoaguChek XS) were obtained from 35 children aged 4 months to 18 years. Mean differences between venous and capillary INR values and their limits of agreement were -0.04 (-0.63 to 0.55) overall, 0.006 (-0.63 to 0.65) for the CoaguChek S and -0.13 (-0.57 to 0.31) for the CoaguChek XS. The Pearson correlation coefficients were 0.88 overall, 0.84 for the CoaguChek S and 0.95 for the CoaguChek XS. Expanded and narrow agreements for all patients were 97.6 and 94%, respectively. In conclusion, home INR self-monitoring is accurate for children requiring long-term oral anticoagulation therapy. Our data suggest that INR self-monitoring with the newer CoaguChek XS is more accurate than with the older CoaguChek S monitor.

Preserved cardiac synchrony and function with single-site left ventricular epicardial pacing during mid-term follow-up in paediatric patients.

AIMS: Right ventricular (RV) pacing may cause dyssynchronous left ventricular (LV) contraction and systolic dysfunction. Left ventricular-based pacing may prevent such deterioration. The aim of this study was to evaluate ventricular synchrony and function with permanent LV pacing (LVP) vs. RV pacing (RVP) in paediatric patients with normal cardiac anatomy. METHODS AND RESULTS: Twenty-five paediatric patients with normal cardiac anatomy and single-site epicardial RV apex pacing (RVP, n=10, pacing duration: 7.9+/-2.9 years) or LV free wall pacing (LVP, n=15, pacing duration: 4.3+/-2.6 years) for complete heart block were enrolled. A total of 15 healthy children served as a control group. Conventional echocardiography, myocardial circumferential (LV), and longitudinal (RV) 2D strain (2Ds) analysis were obtained. Paced QRS duration did not differ between groups (P=0.915). Interventricular mechanical delay (LVP: 17+/-10, RVP: 62+/-15 ms; P<0.0001), septal-to-posterior wall motion delay (LVP: 59+/-23, RVP: 294+/-84 ms; P<0.0001), septal-to-lateral wall motion delay (LVP: 40+/-19, RVP: 59+/-12 ms; P=0.009), and LV mechanical delay (LVP: 35+/-9, RVP: 63+/-17 ms; P<0.0001) were preserved for LVP but not for RVP. Right ventricular mechanical delay was similar among paced groups (P=0.639). Left ventricular ejection fraction was normal for LVP but not for RVP (LVP: 60+/-6%, RVP: 45+/-6%; P=0.012). Left ventricular pacing did not differ from controls for synchrony or function. CONCLUSION: Conventional and 2Ds echocardiographic measurements indicate preserved LV synchrony and function in paediatric patients with LVP compared with RVP. Permanent LVP has no impact on RV synchrony.

Evaluation of the Aesculon cardiac output monitor by subxiphoidal Doppler flow measurement in children with congenital heart defects.

BACKGROUND AND OBJECTIVE: To evaluate the noninvasive electrical velocimetry (Aesculon) monitor for cardiac output (CO) by subxiphoidal Doppler flow measurement in children. METHODS: CO was determined at the end of diagnostic or interventional cardiac catheterization for congenital heart defects. Standard ECG surface electrodes were attached in a vertical direction to the patients' left middle and lower neck, and lower thorax at the level of the heart and xiphoid process. Aesculon CO data were compared with a simultaneously measured CO by the subxiphoidal Doppler flow measurement technique. For each patient, measurements were repeated three times within 5 min. Whitney U-test, simple regression and Bland-Altman analysis were performed to compare CO values obtained by the two techniques. Data are given as range (median). RESULTS: A total of 36 children aged 5.7 (0.5-16.0) years were investigated. CO values obtained by Aesculon monitor [0.55-5.58 (2.62) l min] and subxiphoidal Doppler flow measurements [0.62-6.27 (3.05) l min] differed significantly between both methods (P = 0.04). Simple regression analysis revealed moderate correlation between CO values obtained from the two techniques (r = 0.5544, P < 0.001). Bias between the two methods was 0.31 l min with a precision of 1.92 l min. CONCLUSION: We conclude that electrical velocimetry using the Aesculon monitor does not reliably reflect absolute CO values as compared with subxiphoidal Doppler flow measurement.

Psychological adjustment and quality of life in children and adolescents following open-heart surgery for congenital heart disease: a systematic review.

BACKGROUND: Children with congenital heart defects (CHD) requiring open-heart surgery are a group at high risk for health-related sequelae. Little consensus exists regarding their long-term psychological adjustment (PA) and health-related quality of life (QoL). Thus, we conducted a systematic review to determine the current knowledge on long-term outcome in this population. METHODS: We included randomized controlled trials, case control, or cohort studies published between 1990-2008 evaluating self- and proxy-reported PA and QoL in patients aged between two and 17 years with a follow-up of at least two years after open heart surgery for CHD. RESULTS: Twenty-three studies assessing psychological parameters and 12 studies assessing QoL were included. Methodological quality of the studies varied greatly with most studies showing a moderate quality. Results were as follows: (a) A considerable proportion of children experienced psychological maladjustment according to their parents; (b) studies on self-reported PA indicate a good outcome; (c) the studies on QoL suggest an impaired QoL for some children in particular for those with more severe cardiac disease; (d) parental reports of psychological maladjustment were related to severity of CHD and developmental delay. CONCLUSION: A significant proportion of survivors of open-heart surgery for CHD are at risk for psychological maladjustment and impaired QoL. Future research needs to focus on self-reports, QoL data and adolescents.

Pulse oximetry screening for congenital heart defects in Switzerland: most but not all maternity units screen their neonates.

QUESTIONS: In Switzerland, the screening of neonates for congenital heart defects (CHD) by using pulse oximetry (PO) on the first day of life was recommended in 2005. We assessed the current practice of Swiss maternity units regarding PO screening to define the actual screening rate in Switzerland and to detect possible difficulties in the implementation of this screening. METHODS: This descriptive study was conducted by means of a standardized questionnaire that was sent to all Swiss maternity units. RESULTS: The response rate was 100%. 76% of the units perform regular PO screening and, on the basis of the number of births for 2007, this means that 85% of newborns in Switzerland undergo screening. Although units which do not perform PO screening include every category of maternity unit, the screening is performed to a significantly lower extent in birthing centres than in hospitals (p <0.001). The number of unscreened neonates was 5398 within small units (<1000 births per year) while it was 6137 within large units (>1000 births per year). The main reasons for not performing PO screening are financial factors and the belief that clinical signs are sufficient for the detection of CHD. CONCLUSIONS: Greater effort is needed to implement a nationwide PO screening program for CHD. Special attention should be paid to birthing centres given their limited capacity to treat newborns with critical CHD.

Successful cardiac resynchronization with single-site left ventricular pacing in children.

BACKGROUND: Dyssynchronous left ventricular (LV) contraction due to permanent right ventricular apex (RVA) pacing or delayed electrical activation as typically observed in left bundle brunch block (LBBB) has a negative impact on LV function. Objective was to evaluate the impact of epicardial single-site LV pacing in children on LV function and resynchronization. PATIENTS: Single-site epicardial LV free wall pacing was established in 6 children with congenital heart disease and echocardiographic signs of LV dyssynchrony. Reasons for dyssynchrony were either long-term RVA pacing (n=5; pacing duration: 7.7+/-2.4 years) or LBBB with drug-resistant congestive heart failure (n=1). RESULTS: After 1 month of single-site LV pacing, LV ejection fraction increased (41+/-6 versus 53+/-8%) and LV enddiastolic volume decreased (70+/-22 versus 63+/-18 ml/m(2)) as compared to pre-implant measurements. Interventricular mechanical delay decreased (67+/-15 versus 16+/-15 ms) and intraventricular synchrony was restored (septal-to-posterior wall motion delay: 312+/-24 versus 95+/-57 ms). Accordingly, circumferential 2D strain demonstrated a decrease of LV mechanical delay (201+/-35 versus 99+/-23 ms). CONCLUSION: After 1 month of single-site LV pacing, conventional and 2D strain derived echocardiographic measurements indicated improved ventricular function and synchronization in children with previous RVA pacing or LBBB. Further studies are needed to evaluate whether single-site LV pacing may be sufficient for resynchronization therapy.