http://repub.eur.nl/res/aut/1956/ List of Publications en http://repub.eur.nl/static-eur/img/logo.png http://repub.eur.nl http://repub.eur.nl/res/pub/39706/ 2013-03-19T00:00:00Z Background: Discrete subaortic stenosis is notable for its unpredictable hemodynamic progression in childhood and high reoperation rate; however, data about adulthood are scarce. Methods and Results: Adult patients who previously underwent surgery for discrete subaortic stenosis were included in this retrospective multicenter cohort study. Mixed-effects and joint models were used to assess the postoperative progression of discrete subaortic stenosis and aortic regurgitation, as well as reoperation. A total of 313 patients at 4 centers were included (age at baseline, 20.2 years [25th-75th percentile, 18.4-31.0 years]; 52% male). Median follow-up duration was 12.9 years (25th-75th percentile, 6.2-20.1 years), yielding 5617 patient-years. The peak instantaneous left ventricular outflow tract gradient decreased from 75.7±28.0 mm Hg preoperatively to 15.1±14.1 mm Hg postoperatively (P<0.001) and thereafter increased over time at a rate of 1.31±0.16 mm Hg/y (P=0.001). Mild aortic regurgitation was present in 68% but generally did not progress over time (P=0.76). A preoperative left ventricular outflow tract gradient ≥80 mm Hg was a predictor for progression to moderate aortic regurgitation postoperatively. Eighty patients required at least 1 reoperation (1.8% per patient-year). Predictors for reoperation included female sex (hazard ratio, 1.53; 95% confidence interval, 1.02-2.30) and left ventricular outflow tract gradient progression (hazard ratio, 1.45; 95% confidence interval, 1.31-1.62). Additional myectomy did not reduce the risk for reoperation (P=0.92) but significantly increased the risk of a complete heart block requiring pacemaker implantation (8.1% versus 1.7%; P=0.005). Conclusions: Survival is excellent after surgery for discrete subaortic stenosis; however, reoperation for recurrent discrete subaortic stenosis is not uncommon. Over time, the left ventricular outflow tract gradient slowly increases and mild aortic regurgitation is common, although generally nonprogressive over time. Myectomy does not show additional advantages, and because it is associated with an increased risk of complete heart block, it should not be performed routinely. http://repub.eur.nl/res/pub/37380/ 2012-10-01T00:00:00Z Objectives To evaluate the results of balloon dilatation of stenotic homografts in children, adolescents, and young adults and to identify factors that might influence or predict the effect of the dilatation. Background Homografts are widely used in congenital cardiac surgery; however, the longevity remains a problem mostly because of stenosis in the homograft. The effect of treatment by balloon dilatation is unclear. Methods In a retrospective study, the effect of balloon dilatation was determined by the percentage of reduction of the peak systolic pressure gradient over the homograft during catheterisation and the postponement of re-intervention or replacement of the homograft in months. Successful dilatations - defined in this study as a reduction of more than 33% and postponement of more than 18 months - were compared with unsuccessful dilatations in search of factors influencing or predicting the results. Results The mean reduction of the peak systolic pressure gradient was 30% in 40 procedures. Re-intervention or replacement of the homograft was postponed by a mean of 19 months. In all, 14 balloon dilatations (35%) were successful; the mean reduction was 49% and the mean postponement was 34 months. The time since homograft implantation, the presence of calcification, the homograft/balloon ratio, and the pressure applied during dilatation all tended to correlate with outcome, but were not statistically significant. Conclusions Balloon dilatation is able to reduce the peak systolic pressure gradient over homografts in a subgroup of patients and can be of clinical significance to postpone re-intervention or pulmonary valve replacement. http://repub.eur.nl/res/pub/38672/ 2012-09-26T00:00:00Z Background: The aim of this study was to evaluate the natural progression of aortic dilatation and its association with aortic valve stenosis (AoS) in patients with bicuspid aortic valve (BAV). Methods: Prospective study of aorta dilatation in patients with BAV and AoS using cardiac magnetic resonance (CMR). Aortic root, ascending aorta, aortic peak velocity, left ventricular systolic and diastolic function and mass were assessed at baseline and at 3-year follow-up. Results: Of the 33 enrolled patients, 5 needed surgery, while 28 patients (17 male; mean age: 31 ± 8 years) completed the study. Aortic diameters significantly increased at the aortic annulus, sinus of Valsalva and tubular ascending aorta levels (P < 0.050). The number of patients with dilated tubular ascending aortas increased from 32 % to 43 %. No significant increase in sino-tubular junction diameter was observed. Aortic peak velocity, ejection fraction and myocardial mass significantly increased while the early/late filling ratio significantly decreased at follow-up (P < 0.050). The progression rate of the ascending aorta diameter correlated weakly with the aortic peak velocity at baseline (R2= 0.16, P = 0.040). Conclusion: BAV patients with AoS showed a progressive increase of aortic diameters with maximal expression at the level of the tubular ascending aorta. The progression of aortic dilatation correlated weakly with the severity of AoS. Key Points: • Bicuspid aortic valve (BAV) is the most common congenital heart defect. • BAV patients have an increased risk of developing aortic valve stenosis (AoS). • BAV patients have an increased risk of developing thoracic aorta dilatation. • The severity of aortic stenosis is correlated to the progression of aortic dilatation. • Cardiac magnetic resonance can rapidly assess patients with a bicuspid aortic valve. http://repub.eur.nl/res/pub/33897/ 2011-11-15T00:00:00Z Congenital heart disease (CHD) accounts for nearly one-third of all major congenital anomalies. CHD birth prevalence worldwide and over time is suggested to vary; however, a complete overview is missing. This systematic review included 114 papers, comprising a total study population of 24,091,867 live births with CHD identified in 164,396 individuals. Birth prevalence of total CHD and the 8 most common subtypes were pooled in 5-year time periods since 1930 and in continent and income groups since 1970 using the inverse variance method. Reported total CHD birth prevalence increased substantially over time, from 0.6 per 1,000 live births (95% confidence interval [CI]: 0.4 to 0.8) in 1930 to 1934 to 9.1 per 1,000 live births (95% CI: 9.0 to 9.2) after 1995. Over the last 15 years, stabilization occurred, corresponding to 1.35 million newborns with CHD every year. Significant geographical differences were found. Asia reported the highest CHD birth prevalence, with 9.3 per 1,000 live births (95% CI: 8.9 to 9.7), with relatively more pulmonary outflow obstructions and fewer left ventricular outflow tract obstructions. Reported total CHD birth prevalence in Europe was significantly higher than in North America (8.2 per 1,000 live births [95% CI: 8.1 to 8.3] vs. 6.9 per 1,000 live births [95% CI: 6.7 to 7.1]; p < 0.001). Access to health care is still limited in many parts of the world, as are diagnostic facilities, probably accounting for differences in reported birth prevalence between high- and low-income countries. Observed differences may also be of genetic, environmental, socioeconomical, or ethnic origin, and there needs to be further investigation to tailor the management of this global health problem. http://repub.eur.nl/res/pub/33365/ 2011-07-15T00:00:00Z Recent trials have failed to show that statin therapy halts the progression of calcific aortic stenosis (AS). We hypothesized that statin therapy in younger patients with congenital AS would be more beneficial, because the valve is less calcified. In the present double-blind, placebo-controlled trial, 63 patients with congenital AS (age 18 to 45 years) were randomly assigned to receive either 10 mg of rosuvastatin daily (n = 30) or matched placebo (n = 33). The primary end point was the progression of peak aortic valve velocity. The secondary end points were temporal changes in the left ventricular mass, ascending aortic diameter, and N-terminal pro-brain natriuretic peptide (NT-proBNP). The median follow-up was 2.4 years (interquartile range 1.9 to 3.0). The mean increase in peak velocity was 0.05 ± 0.21 m/s annually in the rosuvastatin group and 0.09 ± 0.24 m/s annually in the placebo group (p = 0.435). The annualized change in the ascending aorta diameter (0.4 ± 1.7 mm with rosuvastatin vs 0.5 ± 1.6 mm with placebo; p = 0.826) and left ventricular mass (1.1 ± 15.8 g with rosuvastatin vs -3.7 ± 30.9 g with placebo; p = 0.476) were not significantly different between the 2 groups. Within the statin group, the NT-proBNP level was 50 pg/ml (range 19 to 98) at baseline and 21 pg/ml (interquartile range 12 to 65) at follow-up (p = 0.638). NT-proBNP increased from 40 pg/ml (interquartile range 20 to 92) to 56 pg/ml (range 26 to 130) within the placebo group (p = 0.008). In conclusion, lipid-lowering therapy with rosuvastatin 10 mg did not reduce the progression of congenital AS in asymptomatic young adult patients. Interestingly, statins halted the increase in NT-proBNP, suggesting a potential positive effect of statins on cardiac function in young patients with congenital AS. http://repub.eur.nl/res/pub/26558/ 2011-07-01T00:00:00Z Background: In tetralogy of Fallot (TOF) pulmonary regurgitation is a frequent complication after initial repair. The objective of the present study was to describe the long-term experience with the use of allograft conduits for right ventricular outflow tract (RVOT) reconstruction after correction of TOF in our institution. Methods: Between 1987 and 2009, 133 allografts were implanted in 126 patients (mean age, 27.8 years). The mean time from initial TOF repair to allograft implantation was 20.8 ± 8.8 years. Kaplan-Meier analyses were done for patient survival, freedom from allograft replacement and freedom from any cardiovascular event. Results: Hospital mortality was 1.5% (2 patients). Mean follow-up was 8.1 years. Ten other patients died during late follow-up, in 8 patients the cause was heart failure. Patient survival was 95% at 5 years, 91% at 10 years, and 80% at 15 years. Male sex, older patient age at the time of operation, and the use of preoperative diuretics were associated with increased risk of mortality during follow-up. Freedom from allograft replacement was 83% at 10 years and 70% at 15 years. Freedom from any valve-related event was 80% at 10 years and 67% at 15 years. Conclusions: Right ventricular outflow tract reconstruction after previous TOF repair can be performed with low risk and a low reintervention rate. Allograft conduits function satisfactorily in the pulmonary position at longer-term follow-up. Functional status after allograft implantation in patients with a previous correction of TOF remains good. There is concern about the long-term survival and the occurrence of heart failure. http://repub.eur.nl/res/pub/28247/ 2010-12-06T00:00:00Z Objective: Allograft conduits are used for reconstruction of the right ventricular outflow tract in congenital heart malformations (biventricular repair) and autograft procedures. A retrospective evaluation of allograft reconstruction of the right-ventricular-outflow-tract reconstruction was conducted and a cross-sectional quality of life study was performed. Methods: Between August 1986 and March 2009, 509 allografts (435 pulmonary and 74 aortic) were implanted in 463 pediatric and adult patients (308 right-sided congenital heart malformations and 155 autograft procedures). Perioperative and follow-up data were collected and analyzed. Kaplan-Meier analyses were done for survival, valve-related re-operation, and valve-related events. Cox regression analysis was used for evaluation of potential risk factors. In addition, the Short Form-36 was presented to patients to assess the perceived quality of life. The results of the Short Form-36 were compared to age-adjusted Dutch population norms. Results: The mean age at allograft implantation was 19 years (1 week-66 years). Mean follow-up was 9 years (2 days-22 years). Forty-eight patients died during follow-up. Patient survival was 93% at 10 years and 88% at 15 years. A total of 63 re-operations were required for allograft dysfunction in 58 patients. Freedom from valve-related re-operation was 89% at 10 years and 81% at 15 years. Freedom from valve-related events was 86% at 10 years and 74% at 15 years. Younger patient age (p = 0.007) and the use of an aortic allograft (p < 0.001) were identified as independent risk factors for allograft re-operation. Patients between 14 and 40 years scored significantly lower on 'physical functioning' and 'general health' subscales than the general Dutch population, but scored better on the subscales 'emotional role functioning' and 'bodily pain'. Except for the subscale 'general health', on which patients within our study population scored lower, patients between 41 and 60 years had comparable average scores as the general Dutch population. The older patient group (61 years or older) had a better average score on the subscale 'bodily pain' and similar scores on other subscales with respect to the general Dutch population. Conclusions: Right-ventricular-outflow-tract reconstruction with an allograft conduit can be performed with good patient survival, acceptable long-term allograft durability, and good perceived quality of life. http://repub.eur.nl/res/pub/24016/ 2010-09-28T00:00:00Z Aim of the study: To investigate the long-term outcome of surgical treatment for congenitally corrected transposition of the great arteries (CCTGA), in patients with biventricular repair with the right ventricle as systemic ventricle.Methods: A total of 32 patients with CCTGA were operated between January 1972 and October 2008. These operations comprised 18 patients with a repair with a normal left ventricular outflow tract, 11 patients with a Rastelli repair of the left ventricle to the pulmonary artery and 3 patients with a cardiac transplantation.Results: Excluding the cardiac transplantation patients, mean age at operation was 16 years (sd 15 years, range 1 week - 49 years). Median follow-up was 12 years (sd 10 years, range 7 days - 32 years). Survival obtained from Kaplan-Meier analysis at 20 years after surgery was 63% (CI 53-73%). For the non-Rastelli group these data at 20 years were 62% (CI 48-76%) and for the Rastelli group 67% (CI 51-83%). Freedom of reoperation at 20 years was 32% (CI 19-45%) in the overall group. In the non-Rastelli group the data at 20 years were 47% (CI 11-83%) and for the Rastelli group 21% (CI 0-54%) after almost 19 years.Conclusions: Long term follow up confirms that surgery in CCTGA with the right ventricle as systemic ventricle has a suboptimal survival and limited freedom of reoperation. Death occurred mostly as a result of cardiac failure. http://repub.eur.nl/res/pub/32992/ 2010-08-01T00:00:00Z http://repub.eur.nl/res/pub/27334/ 2010-07-01T00:00:00Z Transcatheter ablation is an effective method to eliminate the arrhythmogenic substrate in symptomatic children with various types of arrhythmias. A reduction in the procedure and fluoroscopy time would decrease the hazardous effects of the ablation procedures. The magnetic navigation system (MNS) uses atraumatic catheters and facilitates accurate catheter placement in all regions of the heart for mapping and therapy delivery. We compared the efficacy and safety between a manual and MNS-guided approach for mapping and ablation of arrhythmias in a general pediatric arrhythmia population and in a subgroup of young children aged <10 years old. A total of 58 pediatric patients (mean age 12.2 ± 3.2 years) were included in the present study. Of the 58 consecutive patients, 29 were treated with the MNS and 29 underwent conventional manual ablation. No demographic differences were present between the 2 groups. Acute success was achieved in 26 of 29 patients and 27 of 29 patients (p = NS). The mean procedure and fluoroscopy times were comparable in both study groups (168 ± 56 minutes vs 183 ± 52 minutes, p = NS; and 22 ± 59 minutes vs 30 ± 29 minutes, p = NS). In young children (aged <10 years), the success rate did not differ between the 2 groups (10 of 11 vs 6 of 8, p = NS). However, significant decreases in the procedure and fluoroscopy times were achieved (139 ± 57 minutes vs 204 ± 49 minutes and 13 ± 7 minutes vs 31 ± 28 minutes, respectively; p = 0.01 and p = 0.04). In conclusion, our data have strongly suggested that using the MNS for treating young children is advantageous, because it significantly reduced the procedure and fluoroscopy times without compromising efficacy. http://repub.eur.nl/res/pub/27785/ 2010-02-01T00:00:00Z Introduction: Implantable cardioverter defibrillator (ICD) therapy is increasingly used in children. The purpose of this multicenter study is to evaluate mid-term clinical outcome and to identify predictors for device discharge in pediatric ICD recipients. Methods and Results: From 1995 to 2006, 45 patients in The Netherlands under the age of 18 years received an ICD. Mean age at implantation was 10.8 ± 5.2 years. Primary prevention (N = 22) and secondary prevention (N = 23) were equally distributed. Underlying cardiac disorders were primary electrical disease (55%), cardiomyopathy (20%), and congenital heart disease (17%). The follow-up was 44 ± 32.9 months. Three patients (7%) died and one patient (2%) underwent heart transplantation. ICD-related complications occurred in eight patients (17%), seven of whom had lead-related complications. Fourteen patients (31%) received appropriate ICD shocks; 12 patients (27%) received inappropriate ICD shocks. Fifty-five percent of 22 ICD recipients under the age of 12 years received appropriate shocks, which was higher as compared with 9% of 23 older ICD recipients (P = 0.003). Although the incidence of appropriate shocks in the present study was larger in secondary prevention (9/23; 39%) as compared with primary prevention (5/22; 23%), this difference did not reach significance. Conclusions: In our population of patients, children <12 years of age had more appropriate shocks than patients 13-18 years. The complication rate is low, and is mainly lead related. http://repub.eur.nl/res/pub/27100/ 2009-08-01T00:00:00Z http://repub.eur.nl/res/pub/24345/ 2009-07-01T00:00:00Z Objective: With a growing number of children with congenital heart disease (CHD) reaching adulthood, an extensive experience with cardiac surgery in adults with CHD is accumulating. To increase insight in this patient category we report our 17-year single centre experience including predictors for adverse outcome and EuroSCORE performance. Methods: Patients and operative characteristics of all consecutive adult CHD patients operated upon between January 1990 and January 2007 were collected. Categorisation was done according to the EACTS/STS congenital database. Early and late morbidity and mortality were assessed with follow-up extending up to 17 years. EuroSCORE performance was assessed. Results: Nine hundred and sixty-three procedures were performed in 830 patients (mean age 39.3 years, 50.3% male). A total of 49% were re-do procedures, frequent procedures were for left heart lesions (37%), right heart lesions (31%) and septal defects (8%). The 51% primary procedures largely consisted of less complex procedures but also included 1.4% of tetralogy of Fallot repairs, 4.1% of aortic coarctation repairs and 2.7% of Ebstein's disease repairs. Thirty-day mortality was 1.5% (n = 14); predicted mortality by logistic EuroSCORE was 4.6%. c-index was 0.61 (95% CI 0.46-0.75). Major complications such as tamponade requiring intervention occurred in 3.2%, postoperative bleeding requiring re-exploration in 7.1% and renal insufficiency requiring dialysis in 4 (0.4%). Pulmonary hypertension was a strong predictor for short-term mortality; impaired ventricular function and cyanosis for long-term mortality. Overall 17-year survival was 71% (95% CI 61%-82%). Eighty percent of patients were in NYHA class I at last follow-up, 17% in II, 3% in III, 0% in IV. Conclusions: Surgery in adult CHD patients can be performed with low operative mortality and good clinical outcome. EuroSCORE is not a good model for risk assessment in this group of patients. http://repub.eur.nl/res/pub/24872/ 2009-07-01T00:00:00Z Objective: Congenital valvular aortic stenosis is a common congenital heart malformation. The rate of progression in childhood, however, remains to be established. We assessed the progression of peak aortic velocity before intervention as well as the frequency of intervention in paediatric patients with isolated congenital valvular aortic stenosis. Methods: A retrospective cohort study was performed in 245 consecutive patients with aortic stenosis. Both clinical and echocardiographic data were obtained. Results: Over a period of 9.0 (SD 5.2) years (range 0.1-19.4), the mean annual increase in peak systolic velocity was 0.04 m/s/year (95% CI 0.028 to 0.056 m/s/year; p<0.001) as shown by ANOVA. 40 patients underwent a cardiac intervention shortly after their first echocardiogram. Another 33 patients underwent intervention during follow-up. Interventions were performed significantly more often in patients diagnosed at a younger age and/or with a higher peak velocity at diagnosis (p<0.001). Mortality was considerable in those diagnosed in infancy (5-year survival rate of 73% (SD 9%), whereas it was nearly absent in patients diagnosed after infancy. Most patients who died during infancy had progressive left ventricular dysfunction despite adequate relief of left ventricular outflow obstruction. Conclusions: Valvular aortic stenosis in the paediatric age group usually has a good prognosis beyond the neonatal period. Progression over time is usually limited, although a considerable proportion of patients need intervention shortly after initial diagnosis. Mortality, except for the neonatal age group, is nearly absent. http://repub.eur.nl/res/pub/27259/ 2009-07-01T00:00:00Z Results of surgical repair of atrioventricular septal defect (AVSD), both partial (PAVSD) and complete (CAVSD), have improved. However, reoperation is not uncommon. This report describes our experience in 59 patients who underwent reoperation after AVSD repair, between 1977 and 2008. Thirty-one patients had a PAVSD, 28 had a CAVSD. Mean interval between initial repair and reoperation was 10±11 years (PAVSD vs. CAVSD: 13±12 vs. 6±9 years, P=0.063). Reoperations were required for left atrioventricular valve regurgitation (LAVVR) in 53 patients (combined with right atrioventricular valve regurgitation in 10, atrial septal defect (ASD) in 11, ventricular septal defect (VSD) in 7, left ventricular outflow tract (LVOT) obstruction in 1, and aortic valve stenosis in 1), ASD in 3, and LVOT obstruction in 3. Valve repair was performed in 45 patients and replacement in 8. Repair techniques of the leftsided atrioventricular valve (LAVV) included cleft closure in 44 patients, commissuroplasty in 19, and annuloplasty in 1. Freedom from additional reoperation was 85%, and 80% at 5 and 15 years. Hospital mortality was 3%. Overall survival was 91%, and 86% after 5 and 15 years. The most common indication to undergo reoperation is LAVVR. Reoperation is safe and in the majority of cases, a durable repair of the LAVV can still be achieved. http://repub.eur.nl/res/pub/27194/ 2009-03-01T00:00:00Z Background: The improved outcomes and increased availability of surgery for congenital heart disease (CHD) over the last three decades have created a small but steadily increasing subset of patients with unique needs: children and adults with complex arrhythmias in the setting of structural cardiac abnormalities. Radiofrequency catheter ablation (RFCA) in these patients, and in small children with normal cardiac anatomy, is effective but challenging. An understanding of specific anatomical and electrophysiological characteristics of these patients and the technical challenges in addressing them are critical to the success of this therapy. Tools specifically designed for intracardiac diagnosis and therapy in anatomically complex and/or small hearts remain scarce. Aims: We report single-center results from an ongoing registry of all patients with congenital heart disease and all children with complex arrhythmias in which the Magnetic Navigation System (MNS) was used. Results: Included in this report are 12 patients with CHD in whom 17 tachyarrhythmias were treated, and 11 pediatric patients with normal cardiac anatomy who each had a single arrhythmia. The procedures' duration and the duration of fluoroscopy time as well as arrhythmia recurrence rates were comparable to those found in previous reports of procedures performed in adults with structurally normal hearts, and the incidence of complications was quite low. Discussion: In patients with complex congenital malformations, retrograde mapping of the pulmonary venous atrium was feasible, eliminating the need for puncture of the atrial septum, or surgically placed baffle in many cases. Moreover, the design of the catheter eliminated the need for multiple mapping and ablation catheters. Conclusion: Our findings suggest that RFCA using the MNS for arrhythmias after surgery for congenital heart disease and in pediatric patients is safe and effective. http://repub.eur.nl/res/pub/25081/ 2009-01-01T00:00:00Z A baffle puncture is a challenging procedure but can be safely done using direct visualization of the region of interest. To our knowledge, however, it has never been performed in a patient with dextrocardia. We present a 62-year-old male with dextrocardia, right isomerism, congenitally corrected transposition of the great arteries, persistent left-sided superior and inferior caval veins, atrial septum defect, and pulmonary valve stenosis. The atrial septum defect was surgically closed with a Teflon®patch, a variant Mustard operation was performed, and also a prosthetic tricuspid valve was implanted. The patient developed multiple episodes of atrial tachycardia leading to acute heart failure on many occasions. An electrophysiological study was undertaken in order to create a bi-atrial electro-anatomical map. Owing to the presence of a prosthetic tricuspid valve, the femoral venous access was used and a baffle puncture was performed using continuous monitoring with fluoroscopy and transoesophageal echocardiography (TEE). The baffle puncture was successful and the tachycardia was ablated in the systemic venous atrium. To our knowledge, we present the very first case report demonstrating a successful baffle puncture in a patient with dextrocardia and Mustard correction. Direct imaging using TEE seems to be a very useful tool for guiding the puncture. http://repub.eur.nl/res/pub/15905/ 2008-09-01T00:00:00Z Previous studies to determine the sensitivity of the electrocardiogram (ECG) for left ventricular hypertrophy (LVH) in children had their imperfections: they were not done on an unselected hospital population, several criteria used in adults were not applied to children, and obsolete limits of normal for the ECG parameters were used. Furthermore, left ventricular mass (LVM) was taken as the reference standard for LVH, with no regard for other clinical evidence. The study population consisted of 832 children from whom a 12-lead ECG and an M-mode echocardiogram were taken on the same day. The validity of the ECG criteria was judged on the basis of an abnormal LVM index, either alone or in combination with other clinical evidence. The ECG criteria were based on recently established age-dependent normal limits. At 95% specificity, the ECG criteria have low sensitivities (<25%) when an elevated LVM index is taken as the reference for LVH. When clinical evidence is also taken into account, the sensitivity improved considerably (<43%). Sensitivities could be further improved when ECG parameters were combined. The sensitivity of the pediatric ECG in detecting LVH is low but depends strongly on the definition of the reference used for validation. http://repub.eur.nl/res/pub/32333/ 2008-06-01T00:00:00Z http://repub.eur.nl/res/pub/35707/ 2007-11-01T00:00:00Z Cardiomyopathies are classified according to distinct morphological characteristics. They occur relatively frequent and are an important cause of mortality and morbidity. Isolated ventricular non-compaction or non-compaction cardiomyopathy (NCCM) is characterized by an excessively thickened endocardial layer with deep intertrabecular recesses, reminiscent of the myocardium during early embryogenesis. Aims: Autosomal-dominant as well as X-linked inheritance for NCCM has been described and several loci have been associated with the disease. Nevertheless, a major genetic cause for familial NCCM remains to be identified. Methods and Results: We describe, in two separate autosomal-dominant NCCM families, the identification of mutations in the sarcomeric cardiac β-myosin heavy chain gene (MYH7), known to be associated with hypertrophic cardiomyopathy (HCM), restricted cardiomyopathy (RCM), and dilated cardiomyopathy (DCM). Conclusion: These results confirm the genetic heterogeneity of NCCM and suggest that the molecular classification of cardiomyopathies includes an MYH7-associated spectrum of NCCM with HCM, RCM, and DCM. http://repub.eur.nl/res/pub/35749/ 2007-08-01T00:00:00Z Aims: The Ross operation is the operation of choice for children who require aortic valve replacement (AVR) and may also provide a good option in selected adult patients. Although the autograft does not require anticoagulation and has a superior haemodynamic profile, concern regarding autograft and allograft longevity has risen. In this light, we report the 13-year results of our prospective autograft cohort study. Methods and results: Between 1988 and 2005, 146 consecutive patients underwent AVR with a pulmonary autograft at Erasmus Medical Center Rotterdam. Mean age was 22 years (SD 13; range 4 months-52 years), 66% were male. Hospital mortality was 2.7% (N = 4); during follow-up four more patients died. Thirteen-year survival was 94 ± 2%. Over time, 22 patients required autograft reoperation for progressive neo-aortic root dilatation. In addition, eight patients required allograft reoperation. Freedom from autograft reoperation at 13 years was 69 ± 7%. Freedom from allograft reoperation for structural failure at 13 years was 87 ± 5%. Risk factors for autograft reoperation were previous AVR and adult patient age. Conclusion: Although survival of the Rotterdam autograft cohort is excellent, over time a worrisome increase in reoperation rate is observed. Given the progressive autograft dilatation, careful follow-up of these patients is warranted in the second decade after operation. http://repub.eur.nl/res/pub/35497/ 2007-04-01T00:00:00Z Purpose: To prospectively assess, with magnetic resonance (MR) imaging, right ventricular (RV) diastolic function after repair of tetralogy Fallot (TOF) at rest and during pharmacologic stress and to study relationship between main pulmonary artery end-diastolic forward flow (EDFF) (indicative of restrictive RV physiology) and clinical status. Materials and Methods: Institutional medical ethics committee approval and patient or parent informed consent were obtained. Patients with TOF corrected through the transatrial-transpulmonary approach underwent MR imaging at rest and during dobutamine stress and maximal exercise testing. Two-dimensional (2D) cine volumetric data were acquired. Flow measurements were performed with a standard 2D flow-sensitized sequence. MR imaging flow curves for tri-cuspid and pulmonary valves were combined into RV time-volume change curves, from which indexes of RV filling were derived. Patient results were compared with published data in control subjects. Student t tests, Mann-Whitney U tests, analysis of covariance, and paired and one-sample t tests were used. Results: Thirty-six patients (mean age at repair, 0.9 year ± 0.5 [standard deviation]; median age at study inclusion, 17 years, [range, 7-23 years]; 26 male and 10 female patients) were included. Abnormalities in RV filling included impaired relaxation (prolonged deceleration time, P = .002; smaller early filling fraction, P = .02) in the entire group compared with published data in healthy control subjects and signs of restriction to RV filling (smaller atrial filling fraction and higher early filling/atrial filling peak ratio, P < .05 for both) in patients with EDFF (n = 24) compared with patients without EDFF (n = 12). Stress response was abnormal in patients with EDFF, who developed impaired RV relaxation not appreciated at rest. Patients with EDFF had more severe pulmonary regurgitation (P < .05) and poorer exercise performance (P < .001). Conclusion: In patients with TOF corrected with currently widely accepted surgical strategies, pulmonary artery EDFF relates to worse clinical state at mid- to long-term follow-up. Dobutamine stress imaging may unmask abnormalities in RV diastolic filling not appreciated with rest imaging alone. http://repub.eur.nl/res/pub/13649/ 2005-01-01T00:00:00Z We present a case in which LQTS induced severe prenatal and neonatal arrhythmias. LQT3 was diagnosed (mutation R1623Q). Short-acting beta-blockers were ineffective as well as sotalol and mexiletine in preventing recurrent ventricular arrhythmias. An ICD was implanted at the age of 7 months (weight and length of the infant at implantation 6 kg and 60 cm respectively). Flecainide was prescribed in addition to the ICD implantation. After an appropriate shock the flecainide plasma levels were shown to be subtherapeutic. Readjustment of the flecainide dose resulted in adequate plasma levels. No further shocks occurred during a further 17 months follow-up period. The combination of an active can with a subcutaneous patch proved feasible, and lifesaving shocks occurred at 7 months after implantation. http://repub.eur.nl/res/pub/12924/ 2001-07-19T00:00:00Z AIMS: Previous studies that determined the normal limits for the paediatric ECG had their imperfections: ECGs were recorded at a relatively low sampling rate, ECG measurements were conducted manually, or normal limits were presented for only a limited set of parameters. The aim of this study was to establish an up-to-date and complete set of clinically relevant normal limits for the paediatric ECG. METHODS AND RESULTS: ECGs from 1912 healthy Dutch children (age 11 days to 16 years) were recorded at a sampling rate of 1200 Hz. The digitally stored ECGs were analysed using a well-validated ECG computer program. The normal limits of all clinically relevant ECG measurements were determined for nine age groups. Clinically significant differences were shown to exist, compared with previously established normal limits. Sex differences could be demonstrated for QRS duration and several amplitude measurements. CONCLUSIONS: These new normal limits differ substantially from those commonly used and suggest that diagnostic criteria for the paediatric ECG should be adjusted. http://repub.eur.nl/res/pub/9806/ 2001-01-01T00:00:00Z BACKGROUND: Previous studies that determined the frequency content of the pediatric ECG had their limitations: the study population was small or the sampling frequency used by the recording system was low. Therefore, current bandwidth recommendations for recording pediatric ECGs are not well founded. We wanted to establish minimum bandwidth requirements using a large set of pediatric ECGs recorded at a high sampling rate. METHODS AND RESULTS: For 2169 children aged 1 day to 16 years, a 12-lead ECG was recorded at a sampling rate of 1200 Hz. The averaged beats of each ECG were passed through digital filters with different cut off points (50 to 300 Hz in 25-Hz steps). We measured the absolute errors in maximum QRS amplitude for each simulated bandwidth and determined the percentage of records with an error >25 microV. We found that in any lead, a bandwidth of 250 Hz yields amplitude errors <25 microV in >95% of the children <1 year. For older children, a gradual decrease in ECG frequency content was demonstrated. CONCLUSIONS: We recommend a minimum bandwidth of 250 Hz to record pediatric ECGs. This bandwidth is considerably higher than the previous recommendation of 150 Hz from the American Heart Association.