W.A. Helbing

The unnatural history of an atrial septal defect: Longitudinal 35 year follow up after surgical closure at young age

Objective To describe the very long-term outcome after surgical closure of an atrial septal defect (ASD). Design Longitudinal cohort study of 135 consecutive patients who underwent surgical ASD repair at age <15 years between 1968 and 1980. The study protocol included ECG, echocardiography, exercise testing, N-terminal prohormone of brain natriuretic hormone, Holter monitoring and cardiac MRI. Main outcome measures Survival, major events (cardiac reinterventions, stroke, symptomatic arrhythmia or heart failure) and ventricular function. Results After 35 years (range 30–41), survival status was obtained in 131 of 135 patients (97%): five died (4%), including two sudden deaths in the last decade. Fourteen patients (16%) had symptomatic supraventricular tachyarrhythmias and six (6%) had a pacemaker implanted which was predicted by early postoperative arrhythmias. Two reoperations were performed. One ischaemic stroke occurred. Left ventricular (LV) and right ventricular (RV) ejection fractions (EF) were 58±7% and 51±6%, respectively. RVEF was diminished in 17 patients (31%) and in 11 (20%) the RV was dilated. Exercise capacity and quality of life were comparable to the normal population. No clear differences were found between ASD-II or sinus venosus type ASD. Conclusions Very long-term outcome after surgical ASD closure in childhood shows good survival and low morbidity. Early surgical closure prevents pulmonary hypertension and reduces the occurrence of supraventricular arrhythmias. Early postoperative arrhythmias are predictive for the need for pacemaker implantation during early follow-up, but the rate of late pacemaker implantation remains low. Although RVEF was unexpectedly found to be decreased in one-third of patients, the functional status remains excellent.

Maternal smoking during pregnancy, fetal arterial resistance adaptations and cardiovascular function in childhood

Please cite this paper as: Geelhoed J, el Marroun H, Verburg B, van Osch‐Gevers L, Hofman A, Huizink A, Moll H, Verhulst F, Helbing W, Steegers E, Jaddoe V. Maternal smoking during pregnancy, fetal arterial resistance adaptations and cardiovascular function in childhood. BJOG 2011;118:755–762.

Real-Time Transthoracic Three Dimensional Echocardiography: Normal Reference Data for Left Ventricular Dyssynchrony in Adolescents

BACKGROUND Since the introduction of cardiac resynchronization therapy, the assessment of left ventricular (LV) dyssynchrony has become increasingly important. Real-time three-dimensional echocardiography (RT-3DE) is one of the methods that is increasingly used. However, normal reference data in a young population are scarce. METHODS RT-3DE was performed in 73 healthy adolescents aged 12 to 18 years. A systolic dyssynchrony index was derived from the dispersion of time to minimum regional volume for all 16 LV segments. RESULTS Acquisition of RT-3DE data sets was feasible in 67 of 73 subjects (92%). LV end-diastolic volume and LV end-systolic volume were 99+/-25 and 41+/-15 mL, respectively. All controls had highly synchronized segmental function (systolic dyssynchrony index, 1.26+/-0.53%). Dyssynchrony was independent of age, weight, or length. Interobserver variability for the dyssynchrony index was 2+/-2 ms and 0.3+/-0.2% for the absolute and heart rate-corrected values, respectively. Intraobserver variability was 2.0+/-0.07 ms and 0.0+/-0.27%, respectively. CONCLUSIONS RT-3DE could be performed in most healthy adolescents with good interobserver and intraobserver variability. Highly synchronized segmental function was present and was independent of age, weight, or length.

Ventricular function and cardiac reserve in contemporary Fontan patients

BACKGROUND/OBJECTIVE Total cavopulmonary connection (TCPC) has been the preferred treatment for patients with univentricular hearts. Current TCPC-techniques are the intra-atrial lateral tunnel (ILT) and the extracardiac conduit (ECC). We aimed to determine ventricular function during rest and stress, and to compare results for both techniques and for left (LV) versus right ventricular (RV) dominance. METHODS 99 patients, aged 12.5 ± 4.0 years underwent echocardiography and magnetic resonance imaging (MRI), and 69 patients underwent stress MRI. RESULTS Echocardiography showed impaired systolic and diastolic function. MRI parameters were comparable between ILT and ECC at rest. During dobutamine there was a decrease in end-diastolic volume (EDVi) (91 ± 21 vs. 80 ± 20 ml/m(2) p<0.001). Ejection fraction (EF) and cardiac index (CI) during dobutamine were lower for ILT patients (59 ± 11 (ILT) vs. 64 ± 7% (ECC), p=0.027 and 4.2 ± 1.0 (ILT) vs. 4.9 ± 1.0L/min/m(2) (ECC), p=0.006), whereas other parameters were comparable. TEI-index was higher in ILT-patients (0.72 ± 0.27 (ILT) vs. 0.56 ± 0.22 (ECC), p=0.002). Diastolic function was frequently impaired in patients with a dominant RV (67% (RV) vs. 39% (LV), p=0.011). Patients with dominant LVs had smaller end-systolic volume (ESVi) (40 ± 13 (LV) vs. 47 ± 16 (RV) ml/m(2), p=0.030) and higher EF (55 ± 8 (LV) vs. 49 ± 9 ml/m(2) (RV), p=0.001) and contractility (2.3 ± 0.8 (LV) vs. 1.9 ± 0.7 mmHg/ml/m(2) (RV), p=0.050) during rest and higher EF during dobutamine (63 ± 8 (LV) vs. 58 ± 10 ml/m(2) (RV), p=0.043). CONCLUSION Ventricular function is relatively well preserved in modern-day Fontan patients. With dobutamine stress there is a decrease in EDVi. ECC patients have higher CI and EF during stress. Patients with a dominant RV have lower systolic, including impaired contractility, and diastolic function.

Effect of exercise training on sports enjoyment and leisure-time spending in adolescents with complex congenital heart disease: the moderating effect of health behavior and disease knowledge.

OBJECTIVE The aim of this study was to evaluate the effects of a standardized exercise program on sports enjoyment and leisure-time spending in adolescents with congenital heart disease and to know what the moderating impact of their baseline health behavior and disease knowledge is. METHODS Included were 93 patients, aged 10 to 25, with surgical repair for tetralogy of Fallot or with a Fontan circulation for single-ventricle physiology, of 5 participating centers of pediatric cardiology in The Netherlands. They were randomly allocated, stratified for age, gender, and type of congenital heart disease to a 12-week period with either: (1) three times per week standardized exercise training or (2) care as usual (randomization ratio 2:1). At baseline and after 12 weeks, participants completed Web-based questionnaires and were interviewed by phone. OUTCOME MEASURES Primary analyses tested changes from baseline to follow-up in sports enjoyment and leisure-time spending in the exercise group vs. control group. Secondary analyses concerned the moderating influence of baseline health behavior and disease knowledge on changes from baseline to follow-up, and comparison with normative data. RESULTS At follow-up, the exercise group reported a decrease in passive leisure-time spending (watching television and computer usage) compared with controls. Exercise training had no effect on sports enjoyment and active leisure-time spending. Disease knowledge had a moderating effect on improvement in sports enjoyment, whereas health behavior did not. Compared with normative data, patients obtained similar leisure time scores and lower frequencies as to drinking alcohol and smoking. CONCLUSIONS Exercise training decreased passive, but not active, leisure-time spending. It did not influence sports enjoyment.

Enlarged right ventricular size at 11 years' follow-up after closure of secundum-type atrial septal defect in children.

BACKGROUND The fate of right ventricular dimensions after surgical closure of secundum-type atrial septal defects remains unclear. The objectives of this study were to assess ventricular dimensions, exercise capability, and arrhythmias of patients operated for secundum-type atrial septal defect and compare the results with those in healthy references. METHODS A total of 78 consecutive patients underwent surgical closure for a secundum-type atrial septal defect between 1990 and 1995. In all, 42 patients were included and underwent a cross-sectional evaluation including echocardiography, magnetic resonance imaging, exercise testing, and 24-hour ambulatory electrocardiography. Patients were matched with healthy controls for gender, body surface area, and age. RESULTS The mean age at surgery was 4.6 plus or minus 2.8 years, and the mean age at follow-up was 16 plus or minus 3 years. There were no residual intracardiac lesions. The mean right ventricular endsystolic volume was significantly larger in patients (142 ± 26 millilitres) than in references (137 ± 28 millilitres; p = 0.04). In 25% of the patients, right ventricular end-systolic volume was larger than the 95th percentile for references. No relevant arrhythmias were detected. Exercise testing did not reveal differences with healthy references: maximal power (169 ± 43 Watt patients versus 172 ± 53 controls; p = 0.8), maximal oxygen uptake (38 ± 8 versus 41 ± 13 millilitres per minute per kilogram; p = 0.1). COMMENT After surgical closure of secundum-type atrial septal defect, right ventricular end-systolic volume is increased. These findings have no impact on rhythm status or exercise capacity at this stage of follow-up, but may have implications for the timing of surgery or the technique of closure if confirmed in longer follow-up.

Maternal obesity, gestational weight gain and childhood cardiac outcomes: role of childhood body mass index

Background:Maternal obesity may affect cardiovascular outcomes in the offspring. We examined the associations of maternal prepregnancy body mass index and gestational weight gain with childhood cardiac outcomes and explored whether these associations were explained by parental characteristics, infant characteristics or childhood body mass index.Methods:In a population-based prospective cohort study among 4852 parents and their children, we obtained maternal weight before pregnancy and in early, mid- and late pregnancy. At age 6 years, we measured aortic root diameter (cm) and left ventricular dimensions. We calculated left ventricular mass (g), left ventricular mass index (g m−2.7), relative wall thickness ((2 × left ventricular posterior wall thickness)/left ventricular diameter), fractional shorting (%), eccentric left ventricular hypertrophy and concentric remodeling.Results:A one standard deviation score (SDS) higher maternal prepregnancy body mass index was associated with higher left ventricular mass (0.10 SDS (95% confidence interval (CI) 0.08, 0.13)), left ventricular mass index (0.06 SDS (95% CI 0.03, 0.09)) and aortic root diameter (0.09 SDS (95% CI 0.06, 0.12)), but not with relative wall thickness or fractional shortening. A one SDS higher maternal prepregnancy body mass index was associated with an increased risk of eccentric left ventricular hypertrophy (odds ratio 1.21 (95% CI 1.03, 1.41)), but not of concentric remodeling. When analyzing the effects of maternal weight in different periods simultaneously, only maternal prepregnancy weight and early pregnancy weight were associated with left ventricular mass, left ventricular mass index and aortic root diameter (P-values<0.05), independent of weight in other pregnancy periods. All observed associations were independent of parental and infant characteristics, but attenuated to non-significance after adjustment for childhood body mass index.Conclusion:Maternal prepregnancy body mass index and weight gain in early pregnancy are both associated with offspring cardiac structure in childhood, but these associations seem to be fully explained by childhood body mass index.

Cardiac outcome in classic infantile Pompe disease after 13 years of treatment with recombinant human acid alpha-glucosidase

BACKGROUND Cardiac failure is the main cause of death in untreated classic infantile Pompe disease, an inheritable metabolic myopathy characterized by progressive hypertrophic cardiomyopathy. Since the introduction of enzyme replacement therapy (ERT), survival has increased significantly due to reduced cardiac hypertrophy and improved cardiac function. However, little is known about ERTs long-term effects on the heart. METHODS Fourteen patients were included in this prospective study. Cardiac dimensions, function, conduction and rhythm disturbances were evaluated at baseline and at regular intervals thereafter. RESULTS Treatment duration ranged from 1.1 to 13.9 years (median 4.8 years). At baseline, all patients had increased left ventricular mass index (LVMI) (median LVMI 226 g/m2, range 98 to 599 g/m2, Z-score median 7, range 2.4-12.4). During the first four weeks, LVMI continued to increase in six patients. Normalization of LVMI was observed in 13 patients (median 30 weeks; range 3 to 660 weeks). After clinical deterioration, LVMI increased again slightly in one patient. At baseline, PR interval was shortened in all patients; it normalized in only three. A delta-wave pattern on ECG was seen in six patients and resulted in documented periods of supraventricular tachycardias (SVTs) in three patients, two of whom required medication and/or ablation. One patient had severe bradycardia (35 beats/min). CONCLUSION This study shows that ERT significantly reduced LVMI, and sustained this effect over a period of 13.9 years. The risk for rhythm disturbances remains. Regular cardiac evaluations should be continued, also after initially good response to ERT.

Validation of Real-time Transthoracic 3D echocardiography in Children with Congenital Heart Disease

Aims: Assessment of feasibility, accuracy and applicability in clinical practice of real-time three-dimensional echocardiography (RT-3DE) in children with congenital heart defects. Methods and results: From September 2004 to June 2005, 100 consecutive children (57 infants, 43 children > 1 year of age), who were scheduled for corrective intracardiac surgery, were enrolled in this study. RT-3DE was performed with Philips Sonos 7500 echo-system and off-line analysis with TomTec Echoview© software. Quantitative and qualitative assessments of the region of interest were performed on the 3D reconstruction, comparing these results with the anatomic findings and measurements performed during intracardiac surgery. Acquisition of RT-3DE datasets was feasible in 92 of the 100 (92%) patients and acquisition time was 6 ± 3 minutes. The overall quantitative analyses showed an excellent correlation (r < 0.90) between RT-3De and surgery. Also the qualitative analyses were accurate compared with surgical findings, in all patients in which RT-3DE was feasible. Conclusion: This study shows that RT-3DE can be used in the clinical practice for the assessment of intracardiac anatomy in children with congenital heart disease. The information derived from the 3D reconstructions can be taken into consideration in the preoperative planning and management regarding interventional or surgical therapy.

264 Diastolic dysfunction in pediatric renal transplant patients

Eur J Echocardiography Abstracts Supplement, December 2006 and if there were records of the left ventricular inflow by Doppler. Restrictive pattern was defined as E/A >1.5 and E-wave deceleraration time (DTE) 150 ms being pseudonormal if there was left atrium dilation. Results: Echocardiograms of 535 patients were studied, 78% male, aged 64±14. Normal pattern was present in 84 patients (16%), 65 (12%) had abnormal relaxation, 257 (48%) had a pseudonormal pattern and 129 (24%) had restrictive filling pattern. Coronary heart disease was more prevalent in the abnormal relaxation group than in the pseudonormal group (p=0.03). Patients with a restrictive pattern had a higher probability of being hospitalized than those with normal (p=0.001) and pseudonormal patterns (p=0.006). They had higher LVEDV (p=0.0007) and LV end-systolic volume (p<0.0001), lower LV fractional shortening fraction (p<0.0001), higher right ventricle enddiastolic volume (p<0.0001) and bigger left and right atrium dimensions (p<0.0001). A multiple linear regression model (R2=0.114; p<0.0001) determined that right ventricular end-diastolic volume (p=0.0007) was the only independent predictor of DTE. Conclusions: In DCM patients a restrictive filling pattern in a common finding especially in hospitalized patients. It relates to worse LV systolic function and to findings that may traduce higher filling pressures. Right ventricle dimension was the best predictor of the low DTE that characterizes restrictive filling pattern. This finding brings the focus on the possible role of ventricular interaction as a contribution to worse filling of the left ventricle in DCM.