Meijboom, F.J.

Results of balloon dilatation of stenotic homografts in pulmonary position in children and young adults (Article)

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.

Test-retest variability of volumetric right ventricular measurements using real-time three-dimensional echocardiography (Article)

2011-06-01T00:00:00Z

Background: Substantial variability in sequential echocardiographic right ventricular (RV) quantification may exist. Interobserver and intraobserver values are well known, but acquisition (test-retest) variability has been rarely assessed. The objective of this study was to determine the test-retest variability of sequential RV volume and ejection fraction (EF) measurements by real-time three-dimensional echocardiography in patients with congenital heart disease and healthy controls. Methods: Twenty-eight participants (21 patients with congenital heart disease, seven healthy controls; mean age, 30 ± 14 years; 43% men) underwent a series of three echocardiographic studies. To obtain interobserver and intraobserver test-retest variability, two sonographers acquired sequential RV data sets in each participant during one outpatient visit. RV volumetric quantification was done using semiautomated three-dimensional border detection. The variability data were analyzed using correlation coefficients, Bland-Altman analysis, and coefficients of variation. Results: Absolute mean differences for sequential intraobserver acquisitions were 12 ± 12 mL for end-diastolic volume, 7 ± 6 mL for end-systolic volume, and 4 ± 3% for EF. Interobserver and intraobserver test-retest variability, respectively, were 7% and 7% for RV end-diastolic volume, 14% and 7% for end-systolic volume, and 8% and 6% for EF. Conclusions: Good test-retest variability, besides the practical nature of real-time three-dimensional echocardiography for RV volume and EF assessment, makes it a valuable technique for serial follow-up. Although it may be challenging to diminish all factors that can influence echocardiographic examination for serial follow-up, standardization of RV size and functional measurements should be a goal to produce more interchangeable data. Copyright 2011 by the American Society of Echocardiography.

Image orientation for three-dimensional echocardiography of congenital heart disease (Article)

2011-05-31T00:00:00Z

To date there has been little discussion about image orientation for three-dimensional (3D) echocardiography when applied to congenital heart lesions. Anatomic relations cannot be assumed in congenital heart disease and image cropping during post processing may by necessity remove external or even internal anatomic references. We present an approach to consistent anatomic orientation which is both intuitive and consistent with regard to superior-inferior, anterior-posterior and left-right axes. Such anatomic orientation is also concordant with other common 3D imaging modes such as cardiac magnetic resonance imaging and computed tomography. Views derived from standard cross sectional echocardiography have such universal familiarity that analogous 3D projections of these views may be retained but novel hitherto unavailable views such as en face views of the cardiac septums or atrioventricular valves may be projected using anatomic orientation.

Usefulness of real-time three-dimensional echocardiography to identify right ventricular dysfunction in patients with congenital heart disease (Article)

2010-09-15T00:00:00Z

Because right ventricular (RV) dysfunction predicts a poor outcome in patients with congenital heart disease (CHD), regular monitoring of RV function is indicated. To date, cardiac magnetic resonance (CMR) imaging has been the reference method. A more practical, more accessible, and accurate tool would be preferred. We defined normality regarding RV systolic function using healthy controls and tested the ability of real-time 3-dimensional echocardiographic (RT3DE) findings to identify patients with CHD with RV dysfunction. The cutoff values for the RV volumes and ejection fraction (EF) were derived from the CMR imaging findings from 41 healthy controls (mean age 27 ± 8 years, 56% men). In 100 patients with varying CHDs (mean age 27 ± 11 years, 65% men), both RT3DE data sets (iE33) and short-axis CMR imaging (1.5 T) were obtained within 2 hours. The RT3DE and CMR RV volumes and EF were calculated using commercially available software. Receiver operating characteristic curves were created to obtain the sensitivity and specificity of the RT3DE data to identify RV dysfunction. Applying the cutoff values derived from the healthy controls using the CMR data of patients with CHD, we identified 23 patients with an enlarged indexed end-diastolic volume, 29 patients with an enlarged indexed end-systolic volume, and 21 patients with an impaired RVEF. The best cutoff values predicting RV dysfunction using the RT3DE findings were identified (indexed end-diastolic volume >105 ml/m2, indexed end-systolic volume >54 ml/m2, and EF <43%). The RT3DE findings revealed 23 patients with impaired RVEF, with 95% sensitivity, 89% specificity, and a negative predictive value of 99%. In conclusion, real-time 3-dimensional echocardiography is a very sensitive tool to identify RV dysfunction in patients with CHD and could be applied clinically to rule out RV dysfunction or to indicate additional quantitative analysis of RV function.

The emerging burden of hospital admissions of adults with congenital heart disease (Article)

2010-06-01T00:00:00Z

Objective: To assess the extent and the characteristics of hospital admissions in registered adult patients with congenital heart disease. Design: Observational cohort study. Setting: The Netherlands. Patients: 5798 adult patients with congenital heart disease from the Dutch CONCOR national registry linked to the Dutch National Medical Registration (Prismant). Main outcome measures: All hospital admissions from the years 2001 up until 2006. Results: During 28 990 patient-years, 2908 patients (50%) were admitted to hospital. Median age at admission was 39 years (range 18-86 years); 46% were male. Admission rate in CONCOR patients was high among all ages (range 11-68%) and exceeded that of the general Dutch population two to three times; this difference was most pronounced in the older age groups. Altogether there were 8916 admissions, 5411 (61%) of which were for cardiovascular indications. Among cardiovascular admissions, referrals for arrhythmias were most common (31%). Of 4926 interventions, 2459 (50%) were cardiovascular, most often reparative interventions or cardioversion (53%). Most non-cardiovascular admissions were obstetric. Among defects, univentricular heart and tricuspid atresia had the highest incidence and duration of admission. Conclusions: Healthcare utilisation in registered and medically supervised adult patients with congenital heart disease is high and increases with age. Admission rates are at least two times higher than in the general population, and most marked in the older age groups. With the ageing of this population, a major increase in healthcare utilisation is imminent in the near future. Timely preparation of healthcare resources is crucial to sustain optimal care.

Mortality in adult congenital heart disease (Article)

2010-05-01T00:00:00Z

AimsMortality in adults with congenital heart disease is known to be increased, yet its extent and the major mortality risks are unclear.Methods and resultsThe Dutch CONCOR national registry for adult congenital heart disease was linked to the national mortality registry. Cox's regression was used to assess mortality predictors. Of 6933 patients, 197 (2.8) died during a follow-up of 24 865 patient-years. Compared with the general national population, there was excess mortality, particularly in the young. Median age at death was 48.8 years. Of all deaths, 77 had a cardiovascular origin; 45 were due to chronic heart failure (26, age 51.0 years) or sudden death (19, age 39.1 years). Age predicted mortality, as did gender, severity of defect, number of interventions, and number of complications [hazard ratio (HR) range 1.1-5.9, P < 0.05]. Several complications predicted all-cause mortality beyond the effects of age, gender, and congenital heart disease severity, i.e. endocarditis, supraventricular arrhythmias, ventricular arrhythmias, conduction disturbances, myocardial infarction, and pulmonary hypertension (HR range 1.4-3.1, P < 0.05). These risks were similar in patients above and below 40 years of age. Almost all complications predicted death due to heart failure (HR range 2.0-5.1, P < 0.05); conduction disturbances and pulmonary hypertension predicted sudden death (HR range 2.0-4.7, P < 0.05). Conclusion Mortality is increased in adults with congenital heart disease, particularly in the young. The vast majority die from cardiovascular causes. Mortality risk, particularly by heart failure, is increased by virtually all complications. Complications are equally hazardous in younger as in older patients.

Pregnancy outcome in women with repaired versus unrepaired isolated ventricular septal defect (Article)

2010-05-01T00:00:00Z

Objective To compare the risks of pregnancy complications in women with repaired and unrepaired isolated ventricular septal defect (VSD). Design A retrospective multicentre study. Setting Tertiary centres in the Netherlands and Belgium. Methods Women were identified using two congenital heart disease registries. Eighty-eight women were identified who had experienced 202 pregnancies, including 46 miscarriages and nine terminations of pregnancy. Information on each completed pregnancy (n = 147; unrepaired VSD, n = 104; repaired VSD, n = 43) was obtained using medical records and telephone interviews. Data from the Generation R database (prospective cohort study; n = 9667) were used to determine the background risk (controls). Odds ratios and 95% CI were estimated using general estimation equation analysis adjusted for multiple pregnancies per woman, maternal age and parity status. Main outcome measures Adjusted odds ratios (AORs) for developing pregnancy complications in relation to corrective status. Results Pregnancies in women with an unrepaired VSD were associated with a higher risk of pre-eclampsia (AOR 4.59, 95% CI 2.01-10.5, P < 0.001) compared with controls. No differences were observed when comparing women with repaired VSD and controls. Pregnancies in women with repaired VSD were associated with a higher risk of premature labour (AOR 4.02, 95% CI 1.12-14.4, P = 0.03) and small-for-gestational-age (SGA) births (AOR 4.09, 95% CI 1.27-13.2, P = 0.02) compared with women with unrepaired VSD. Conclusions Women with unrepaired VSD are at increased risk of pre-eclampsia, which suggests that it is not a benign condition. In addition, women with repaired VSD are at increased risk of premature labour and SGA births compared with women with unrepaired VSD.

Clinical Value of Real-Time Three-Dimensional Echocardiography for Right Ventricular Quantification in Congenital Heart Disease: Validation With Cardiac Magnetic Resonance Imaging (Article)

2010-02-01T00:00:00Z

Background: The objective of this study was to test the feasibility, accuracy, and reproducibility of the assessment of right ventricular (RV) volumes and ejection fraction (EF) using real-time three-dimensional echocardiographic (RT3DE) imaging in patients with congenital heart disease (CHD), using cardiac magnetic resonance (CMR) as a reference. Methods: RT3DE data sets and short-axis cine CMR images were obtained in 62 consecutive patients (mean age, 26.9 ± 10.4 years; 65% men) with various CHDs. RV volumetric quantification was done using semiautomated 3-dimensional border detection for RT3DE images and manual tracing of contours in multiple slices for CMR images. Results: Adequate RV RT3DE data sets could be analyzed in 50 of 62 patients (81%). The time needed for RV acquisition and analysis was less for RT3DE imaging than for CMR (P < .001). Compared with CMR, RT3DE imaging underestimated RV end-diastolic and end-systolic volumes and EF by 34 ± 65 mL, 11 ± 55 mL, and 4 ± 13% (P < .05) with 95% limits of agreement of ±131 mL, ±109 mL, and ±27%, as shown by Bland-Altman analyses, with highly significant correlations (r = 0.93, r = 0.91, and r = 0.74, respectively, P < .001). Interobserver variability was 1 ± 15%, 6 ± 17%, and 8 ± 13% for end-diastolic and end-systolic volumes and EF, respectively. Conclusion: In the majority of unselected patients with complex CHD, RT3DE imaging provides a fast and reproducible assessment of RV volumes and EF with fair to good accuracy compared with CMR reference data when using current commercially available hardware and software. Further studies are warranted to confirm our data in similar and other patient populations to establish its use in clinical practice.

Comparison of pregnancy outcomes in women with repaired versus unrepaired atrial septal defect (Article)

2009-11-01T00:00:00Z

Objective To compare the risks of complications during pregnancy in women with repaired andunrepaired atrial septal defects (ASDs) without associated complex cardiac lesions. Design A retrospective multicentre study. Setting Tertiary centres in the Netherlands and Belgium. Population Women with ASD without associated complex cardiac lesions. Methods Women were identified using two congenital heart disease registries. One hundred women were identified who had 243 pregnancies, including 49 miscarriages and six terminations of pregnancy. Detailed information on each completed pregnancy (n = 188; unrepaired ASD, n = 133; repaired ASD, n = 55) was obtained using medical records and telephone interviews. In addition, data from the Generation R database (a prospective cohort study; n = 9667) were used to determine the background risk (control group). Main outcome measures Adjusted odds ratios (AORs) for cardiac, obstetric and neonatal events controlled for multiple pregnancies per woman using general estimating equation analysis. Results Women with an unrepaired ASD had a higher risk of neonatal events (AOR = 2.99, 95% confidence interval [CI] 1.14-7.89, P = 0.027) than women with a repaired ASD. The risk of cardiac and obstetric complications was comparable between women with unrepaired and repaired ASDs. Compared with the general population, women with an unrepaired ASD had higher risks of pre-eclampsia (AOR = 3.54, 95% CI 1.26-9.98, P = 0.017), small-for-gestational-age births (AOR = 1.95, 95% CI 1.15-3.30, P = 0.013) and fetal mortality (AOR = 5.55, 95% CI 1.77-17.4, P = 0.003). By contrast, no differences were observed when comparing women with a repaired ASD versus controls. Conclusions Women with an unrepaired ASD are at increased risk of neonatal events in comparison with women with a repaired ASD. Compared with the general population, women with an unrepaired ASD are at increased risk of pre-eclampsia, small-for-gestational-age births and fetal mortality.

Seventeen years of adult congenital heart surgery: a single centre experience (Article)

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.

Assessment of biventricular functional reserve and NT-proBNP levels in patients with RV volume overload after repair of tetralogy of Fallot at young age (Article)

2009-04-17T00:00:00Z

Purpose: To assess biventricular functional reserve (FR), NT-proBNP levels and exercise performance, in relation to right ventricular volume in patients with pulmonary regurgitation (PR) after repair of tetralogy of Fallot (TOF) at young age. Methods: In 53 TOF patients (maximum age at repair 2.0 years, interval since repair 15 (5) years) without residual lesions except PR, biventricular FR (derived from magnetic resonance imaging with dobutamine stress), NT-proBNP levels, maximal workload, and peak oxygen uptake were assessed. Results: Mean right ventricular end-diastolic volume was 140(38) ml/m2. Median pulmonary regurgitant fraction was 37% (range 0-57%). Biventricular systolic stress response was normal: mean (SD) ESV decreased (ΔRVESV - 17(8) ml/m2, ΔLVESV - 11(5)), SV increased (ΔRVSV + 12(9) ml/m2, ΔLVSV + 9(6)), FR was positive in all (RV-FR + 11(5)%, LV-FR + 13(6)). No serious adverse effects to dobutamine were encountered. NT-proBNP was increased in 2 patients. Median level was 10 pmol/L (range 2-42). NT-proBNP correlated with PR-percentage but not with right ventricular size. High-risk levels of NT-proBNP indicated a smaller RV-FR and a smaller decrease of biventricular ESV. Mean (SEM) VO2maxwas 96(3)%, mean Workloadmax89(2)% of predicted. Conclusion: At mid to long term follow-up overall NT-proBNP levels are normal and biventricular functional reserve and exercise tolerance are well preserved in TOF repaired at young age, irrespective of RV volume. This questions the validity of isolated PR or RV volume criteria for pulmonary valve replacement in this group. Low-dose dobutamine stress testing is well tolerated and may be a useful additional tool for clinical decision making.

Double orifice mitral valve by real-time three-dimensional echocardiography (Article)

2008-09-01T00:00:00Z

Double orifice mitral valve (DOMV) is a rare congenital malformation described as division of mitral orifice into two anatomically distinct orifices separated by an accessory bridge of fibrous tissue. In 85% of cases, both orifices are unequal in size. It is usually associated with other congenital defects such as atrioventricular septal defect and complex congenital heart disease. Most of cases could be diagnosed by two-dimensional echocardiography (2DE). The real-time three-dimensional echocardiography (RT3DE) helped in more detailed structure and function. Presented here RT3DE used for orientation of DOMV that allowed detailed and comprehensive assessment incremental to that obtained by 2DE.

Abnormal aortic elastic properties in adults with congenital valvular aortic stenosis (Article)

2008-08-29T00:00:00Z

Background: Abnormalities of the aortic root are common in patients with a bicuspid aortic valve. Our aim was to investigate the elastic properties of the aortic root in patients with congenital aortic valvular stenosis (AS) in comparison with age- and gender-matched controls, and to investigate the influence of stenosis severity and aortic size on aortic root elasticity. Methods: Thirty-two adults (mean age 30.4 ± 7.5 years, 22 men) with congenital AS without previous cardiovascular surgery were prospectively studied. Aortic root elasticity indices such as aortic stiffness index (ASI), aortic root distensibility (ARD), and aortic strain were calculated with the use of M-mode echocardiography. Results: ASI was significantly higher in patients compared to controls, 8.5 ± 8.4 versus 4.0 ± 1.4, respectively (P < 0.01). Other indices of aortic root elasticity were similar between patients and controls: ARD was 4.2 ± 3.6 versus 4.3 ± 1.9 × 10- 6cm2/dynes, respectively, and aortic strain was 12.4 ± 9.6 versus 13.5 ± 5.0%, respectively (P = NS for all). Correlations were found between aortic size and indices of aortic elasticity (i.e., aortic strain and ARD), denoting that an increased aortic dimension is associated with a stiffer aorta. Interestingly, no correlations were found between indices of severity of AS and aortic elasticity, suggesting that an abnormal aortic elasticity is independent of stenosis severity. Conclusions: Congenital AS results in abnormal aortic elastic properties, independent of stenosis severity. Furthermore, there seems to be a relationship between aortic dimensions and aortic stiffness.

Long-term follow-up after repair of Ebstein's anomaly (Article)

2008-07-01T00:00:00Z

Objective: We studied the long-term results of vertical plication repair of Ebstein's anomaly according to Carpentier. Methods: Between 1988 and 2007, 28 patients (mean age 28.8 ± 15.7 years, range 4-58 years) underwent vertical plication repair of Ebstein's anomaly. At operation the anomaly was classified according to Carpentier. In three patients (11%) a cavopulmonary shunt was added at the repair on the indication of impaired right ventricular function. Results: There was no operative mortality. Early mortality was 3.6% (one patient). Actuarial survival and actuarial freedom from reoperation at 19 years were 96% (95% CI; 96-97%) and 72% (95% CI; 53-92%), respectively. Six patients required reoperation, with a successful re-repair in three patients. Mean duration of follow-up was 10.7 ± 6.5 years. One year postoperatively, tricuspid incompetence had decreased significantly (p < 0.001), as had New York Heart Association (NYHA) functional class (p < 0.001). In addition, exercise tolerance had increased (70 ± 19% to 92 ± 9% of predicted values, p < 0.05). Both tricuspid function and NYHA functional class remained essentially unchanged at the end of follow-up, indicating durable haemodynamic and functional results. Conclusion: This study demonstrates favourable long-term results following vertical plication repair of Ebstein's anomaly with low mortality, acceptable morbidity and good haemodynamic and functional results.

Repaired and open atrial septal defects type II in adulthood: An epidemiological study of a large European cohort (Article)

2008-06-06T00:00:00Z

Background: There is a lack of evidence regarding treatment options for adults with an atrial septal defect (ASD) who present with an open defect or with sequelae after closure of the defect. The aim of this study was to describe the clinical characteristics and treatment of a large cohort of adult patients born with an ASD type II. Methods and results: Data on the clinical characteristics of 882 ASD II patients (mean follow-up of 4.2 years) included in the Euro Heart Survey on adult congenital heart disease were analysed. At baseline, the defects of 377 patients (mean age 39.2 (16.1) years; 65% females) had been closed, leaving 505 patients (mean age 41.1 (16.4) years; 68% females) with an open ASD. Hemodynamic abnormalities were more prevalent among patients with an open compared to those with a closed defect at baseline: pulmonary arterial hypertension 35% versus13%; right ventricular (RV) dysfunction 31% versus 8%; and severe RV volume overload 18% versus 1% (all P-values < 0.001). These prevalences increased with age, but hemodynamic parameters remained stable during follow-up in nearly all patients with a small defect. Also functional limitations were more common in those with open defects at baseline compared to those with closed defects (54% versus 25%). There was no difference in the prevalence of arrhythmia's. The best independent predictors of functional limitations appeared to be PAH (odds ratio 25.2 (5.8-109.6); P < 0.001)) and RV volume overload (odds ratio 2.3 (1.5-3.4; P < 0.001)) in a multivariable model. During follow-up, 9 patients died and in 294 patients the defect was closed, in 180 patients surgically, and in 114 patients by device. Among the latter group there were relatively more females (78% vs 66%; P = 0.035). In the surgically closed group defects were more "severe". There were substantial differences according to country in the relative frequency of device closure versus surgical closure, as well as the size and hemodynamic severity of the defects closed. Conclusion: The data from this study provide a cross-section of the kind of adult patients with an ASD that are seen at outpatient clinics for adult congenital heart disease throughout Europe. Taken together, non-operated patients fared significantly worse in all aspects of hemodynamics studied than the patients whose defects had been closed. In moderate or large defects, when not-operated, clinical parameters tend to worsen with time, and closure of such a defect-the sooner the better-seems always to be the preferred treatment option. In the majority of small defects, operation is not necessarily indicated.

Usefulness of Cardiac Magnetic Resonance Imaging Combined With Low-Dose Dobutamine Stress to Detect an Abnormal Ventricular Stress Response in Children and Young Adults After Fontan Operation at Young Age (Article)

2008-06-01T00:00:00Z

After Fontan operation, patients are limited in increasing cardiac output and in exercise capacity. This has been related to impaired preload or other factors leading to decreased global ventricular performance with stress. To study these factors, the stress responses of functionally univentricular hearts were assessed at rest and during low-dose dobutamine stress using cardiovascular magnetic resonance imaging. Thirty-two patients after Fontan completion at young age were included (27 with total cavopulmonary connection, 5 with atriopulmonary connection; mean age 13.3 years, range 7.5 to 22.2; 23 male patients; median follow-up after Fontan operation 8.1 years, range 5.2 to 17.8). A multiphase short-axis stack of 10 to 12 contiguous slices of the systemic ventricle was obtained at rest and during low-dose dobutamine stress cardiovascular magnetic resonance imaging (maximum 7.5 μg/kg/min). With stress-testing, heart rate, ejection fraction, and cardiac index increased adequately (p <0.001). There was an abnormal decrease in end-diastolic volume and an adequate decrease in end-systolic volume (p <0.001). Stroke volume did not change with stress testing (p = 0.15). At rest, dominant left ventricles had higher ejection fractions than dominant right ventricles (p = 0.01), but this difference disappeared with stress testing. In conclusion, a functionally univentricular heart after Fontan completion at young age has an adequate increase in ejection fraction with β-adrenergic stimulation. However, as a result of impaired preload with stress, cardiac output can be increased only by increasing heart rate.

Risk of complications during pregnancy in women with congenital aortic stenosis (Article)

2008-05-23T00:00:00Z

Background: Pregnancy in women with congenital aortic stenosis (AS) is associated with increased cardiac complications. Data on non-cardiac complications are limited, and this information is crucial for prenatal counselling and perinatal care. The aim of this study was to present the maternal and perinatal outcome of pregnancy in women with congenital AS. Methods: By review of the Dutch CONCOR national registry and a local Belgian tertiary care centre database, 35 women with congenital AS with a history of completed pregnancy before aortic valve replacement were enrolled in this study. Medical history and maternal and perinatal outcome were determined. Results: Thirty-five women had 58 pregnancies resulting in 53 successful pregnancies, three miscarriages, and two abortions. The most serious cardiac complications were heart failure (n = 2, 3.8%) and atrial arrhythmia (n = 3, 5.7%). Although cardiac complications were present (9.4%), obstetric (22.6%) and perinatal (24.5%) complications were observed more often. A total of six pregnancies (11.3%) were complicated by hypertension-related disorders, including one case of eclampsia. Furthermore, 7 premature births (13.2%) and 7 small-for-gestational-age births (13.2%) were encountered. Pregnancy in women with severe AS was characterized by an increased incidence of heart failure and premature labour, and shorter pregnancy duration. Older women (> 30 years) were at increased risk of perinatal events (odds ratio 4.38, 95% confidence interval 1.02 to 18.81). Conclusions: Pregnancy is generally well tolerated in women with congenital AS. Importantly, an excess of obstetric and perinatal complications was found, requiring more meticulous attention.

Steepened aortoseptal angle may be a risk factor for discrete subaortic stenosis in adults (Article)

2008-05-07T00:00:00Z

Discussion exists whether discrete subaortic stenosis (DSS) is a congenital or acquired cardiac defect. Currently, it is regarded an "acquired" cardiac defect presumably secondary to altered flow patterns due to morphological abnormalities in the left ventricular outflow tract, as have been shown by some studies in the pediatric population. In this report, we demonstrated a steepened aortoseptal angle in adults with DSS without previous cardiac surgery in comparison to controls. Our results strengthen the hypothesis that altered flow patterns due to a steepened aortoseptal angle are a substrate for development of DSS in adults.

Changes during exercise of ECG intervals related to increased risk for ventricular arrhythmia in repaired tetralogy of Fallot and their relationship to right ventricular size and function (Article)

2008-03-14T00:00:00Z

Purpose: Our study aimed to assess pro-arrhythmogenic electrocardiographic changes during maximal physical exercise in patients operated for Tetralogy of Fallot (TOF). Methods: TOF patients prospectively underwent: 1) bicycle ergometry, 2) cardiac MRI, and 3) 24-hour Holter. ECG data was analyzed at rest, at 60% of peak exercise and at peak exercise. R-R duration, QRS-, QT- and JT-duration and dispersions were assessed. Changes of ECG parameters during exercise were calculated and correlated to RV volume, RVEF, RV wall-mass, PR-percentage and VO2max. Exercise ECG data from healthy controls were used as reference. Results: Thirty-one patients (mean age at repair (SD) 0.8 (0.5) years, age at study 16 (5) years) and 25 controls (age 12 (2) years) were included. With exercise mean QTc and JTc dispersions increased in patients (p < 0.001), but not in controls. At peak exercise JTc dispersion was larger in patients (p < 0.01). QTc did not change with exercise in patients (p = 0.14) and decreased in controls (p < 0.05). At all levels of exercise mean QTc, QRS and QRS dispersion were larger in patients (all p < 0.001). Significant associations were found for; 1) a larger increase of JTc dispersion with a higher PR-percentage, a larger RV volume, a larger RV wall-mass, 2) a larger QTc increase with a larger RV volume and worse RVEF. Conclusion: During physical exercise inhomogeneity of repolarisation, known to predispose for re-entry ventricular arrhythmia, increases in repaired TOF. Larger inhomogeneity is found with more severe PR.

Rapid and accurate measurement of LV mass by biplane real-time 3D echocardiography in patients with concentric LV hypertrophy: Comparison to CMR (Article)

2008-03-01T00:00:00Z

Aims: To evaluate the accuracy of real-time three-dimensional echocardiography (RT3DE) using a biplane and multiplane method in determining left ventricular (LV) mass compared to cardiac magnetic resonance imaging (CMR). Methods and results: LV mass was measured in 18 adult patients with congenital aortic stenosis using CMR and echocardiography (M-mode, two-dimensional echocardiography (2DE), and RT3DE). RT3DE data were analysed using a biplane and multiplane method. No geometric assumptions were necessary using the multiplane RT3DE method.With regard to biplane or multiplane RT3DE, no tendency of over- or underestimation of LV mass was observed. Pearson's correlation coefficients for RT3DE versus CMR were 0.84 and 0.90 for the biplane and multiplane method, respectively. In addition, the accuracy of both RT3DE methods were comparable (Fisher's R-to-Z transformation: Z = 0.69, P = NS). Finally, off-line analysis using biplane RT3DE was significantly faster than multiplane RT3DE (3.8 ± 1.2 vs. 7.8 ± 1.7 minutes, P < 0.001). Conclusions: Biplane RT3DE provided an accurate estimate of LV mass in patients with concentric left ventricular hypertrophy, which was not improved by multiplane RT3DE. The accuracy and speed of analysis renders biplane RT3DE an attractive tool in daily clinical practice for assessing the degree of LV hypertrophy.

Consequences of a selective approach toward pulmonary valve replacement in adult patients with tetralogy of Fallot and pulmonary regurgitation (Article)

2008-01-01T00:00:00Z

Objective: The aim of the study was to assess the long-term results of a selective policy toward pulmonary valve replacement in adult patients with repaired tetralogy of Fallot and severe pulmonary regurgitation. Methods: Sixty-seven patients with tetralogy of Fallot were followed up from 15 ± 3 years until 27 ± 3 years after surgery. Results: Twenty-two patients had mild-to-moderate pulmonary regurgitation. No significant changes occurred in the follow-up period. Of 45 patients with severe pulmonary regurgitation and severe right ventricular dilatation, 28 (62%) remained free of symptoms and did not undergo pulmonary valve replacement. No changes in right ventricular size or exercise capacity were found. In 3 (11%) of 28 patients, QRS duration increased to more than 180 ms. Seventeen patients had symptoms and underwent pulmonary valve replacement: 9 (54%) of 17 patients improved clinically and echocardiographically, and QRS duration shortened postoperatively. Right ventricular dimensions did not regress despite pulmonary valve replacement in 8 patients. Conclusion: Refraining from pulmonary valve replacement in asymptomatic patients led to no measurable deterioration in 25 (89%) of 28 patients. Referring symptomatic patients for pulmonary valve replacement led to an improvement in 9 (53%) of 17 patients. In 11 (24%) of 45, a selective approach led to questionable or unsatisfactory results.

Congenital aortic stenosis in adults: Rate of progression and predictors of clinical outcome (Article)

2007-11-30T00:00:00Z

Background: Little data are available on the natural history of young adults with congenital valvular aortic stenosis (AS). The aim of the present study was to determine the progression rate of AS in young adults, and to identify predictors of stenosis progression and outcome. Methods: Retrospective study of all patients seen at a single centre diagnosed with congenital AS (≥ 2.5 m/s) between 1992 and 2005, excluding patients with severe aortic regurgitation. The slope of the regression of the aortic jet velocity on the time elapsed since the baseline study was used to define the rate of progression of stenosis. Results: A total of 84 adults (mean age, 23.5 ± 7.9 years) were studied who had at least two echocardiograms > 1 year (5.6 ± 2.6 years) apart. The annual progression of aortic jet velocity was 0.09 ± 0.15 m/s per year. Multivariable linear regression analysis identified older age (p < 0.001) as an independent predictor of faster haemodynamic progression. During the follow-up period of 7.7 ± 2.7 years, no patient died and 35 patients (42%) underwent aortic valve intervention. By multivariable Cox regression analysis, severe AS (≥ 4.0 m/s) and rapid progression of aortic jet velocity (≥ 0.2 m/s/year) were independent predictors of intervention. Cumulative intervention-free survival for patients with severe AS was 78 ± 8% at 3 years and 48 ± 10% at 5 years versus respectively 98 ± 2% and 96 ± 3% for patients with mild-to-moderate AS (log-rank: p < 0.001). Conclusions: Progression of congenital AS was relatively low in young adults compared to elderly with degenerative AS. Older age was associated with more rapid progression.

Long-term follow-up and quality of life after closure of ventricular septal defect in adults (Article)

2007-08-01T00:00:00Z

Objective: To study patients who underwent surgical closure of a congenital ventricular septal defect (VSD) and presenting at adult age. Methods and results: A retrospective study was carried out of 28 patients (15 male) operated upon between 1980 and 2004. Patients were investigated by echocardiography, ECG and assessed for quality of life by a questionnaire. The indication for surgery was volume overload in 11 patients, endocarditis in 8, aortic valve regurgitation in 8 and the combination of a VSD with subvalvular aortic stenosis in 1. Follow-up was complete with a mean duration of follow-up of 13 years. There was no early or late mortality. One patient was reoperated for recurrent VSD. Twenty-five patients underwent echocardiography, which revealed a trivial residual VSD in two and mild aortic regurgitation in 10 (40%) patients. One patient was in atrial fibrillation. Health related quality of life in the dimensions cognitive functioning and sleep differed significantly from that of the general population. Conclusion: With a relative difference in indications for closure of a VSD in adulthood, surgical closure of VSD at adult age is an adequate and safe procedure, with good results on long-term follow-up. Progression of aortic valve regurgitation is a matter of concern.

Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance (Article)

2007-07-01T00:00:00Z

BACKGROUND - To facilitate the optimal timing of pulmonary valve replacement, we analyzed preoperative thresholds of right ventricular (RV) volumes above which no decrease or normalization of RV size takes place after surgery. METHODS AND RESULTS - Between 1993 and 2006, 71 adult patients with corrected tetralogy of Fallot underwent pulmonary valve replacement in a nationwide, prospective follow-up study. Patients were evaluated with cardiovascular magnetic resonance both preoperatively and postoperatively. Changes in RV volumes were expressed as relative change from baseline. RV volumes decreased with a mean of 28%. RV ejection fraction did not change significantly after surgery (from 42±10% to 43±10%; P=0.34). Concomitant RV outflow tract reduction resulted in a 25% larger decrease of RV volumes. After correction for surgical RV outflow tract reduction, higher preoperative RV volumes (mL/m) were independently associated with a larger decrease of RV volumes (RV end-diastolic volume: β=0.41; P<0.001). Receiver operating characteristic analysis revealed a cutoff value of 160 mL/m for normalization of RV end-diastolic volume or 82 mL/m for RV end-systolic volume. CONCLUSIONS - Overall, we could not find a threshold above which RV volumes did not decrease after surgery. Preoperative RV volumes were independently associated with RV remodeling and also when corrected for a surgical reduction of the RV outflow tract. However, normalization could be achieved when preoperative RV end-diastolic volume was <160 mL/m or RV end-systolic volume was <82 mL/m.

The use of contrast echocardiography for the detection of cardiac shunts (Article)

2007-06-01T00:00:00Z

Recently, debate has erupted about the clinical significance of cardiovascular shunts. Several major health problems such as stroke and migraine have been associated with patent foramen ovale (PFO) with right-to-left shunt (RLS). The nature of the relationship between these syndromes and PFO is not clearly understood. Technical advances have led to more therapeutic options including device closure of PFO, hence prevention of such a PFO-related stroke has become feasible. Therefore, optimal diagnosis of PFO has become of greater clinical importance. Contrast echocardiography with non-transpulmonary contrast agents has been the cornerstone in diagnosis of PFO with RLS for over four decades. Despite being a relatively invasive procedure, transesophageal echocardiography (TEE) is considered the gold standard for detection of RLS. Several other echocardiographic techniques such as transthoracic echocardiography (TTE) with second harmonic imaging and transcranial Doppler ultrasonography (TCD) have shown increased sensitivity and specificity compared to TEE for the detection of PFO with RLS. Moreover, improvement of skills and techniques used for detection of these shunts has led to greater detection of small and large sized RLS in the echocardiographic laboratory. This review gives and overview of the echocardiographic techniques, contrast agents and manoeuvres used for detection of the major cardiovascular shunts and their clinical relevance to major health problems.

Diastolic function in repaired tetralogy of fallot at rest and during stress: Assessment with MR imaging (Article)

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.

Assessment of pulmonary valve and right ventricular outflow tract with real-time three-dimensional echocardiography (Article)

2007-04-01T00:00:00Z

Aim: Assessment of pulmonary valve (PV) and right ventricular outflow tract (RVOT) using real-time 3-dimensional echocardiography (RT3DE). Methods: Two-dimensional echocardiography (2DE) and RT3DE were performed in 50 patients with congenital heart disease (mean age 32 ± 9.5 years, 60% female). Measurements were obtained at parasternal views: short axis (PSAX) at aortic valve level and long axis (PLAX) with superior tilting. RT3DE visualization was evaluated by 4-point score (1: not visualized, 2: inadequate, 3: sufficient, and 4: excellent). Diameters of PV annulus (PVAD), and RVOT (RVOTD) were measured by both 2DE and RT3DE, while areas (PVAA) and (RVOTA) by RT3DE only. Results: By RT3DE, PV was visualized sufficiently in 68% and RVOTexcellently in 40%. PVAD and PVAA were measured in 88%. RVOTD and PVAD by 2DE at PLAX were significantly higher than PSAX (P < 0.0001) and lower than that by RT3DE (P < 0.001). Conclusion: RT3DE helps inRVOT and PV assessment adding more details supplemental to 2DE.

Virtual reality 3D echocardiography in the assessment of tricuspid valve function after surgical closure of ventricular septal defect (Article)

2007-03-12T00:00:00Z

Background. This study was done to investigate the potential additional role of virtual reality, using three-dimensional (3D) echocardiographic holograms, in the postoperative assessment of tricuspid valve function after surgical closure of ventricular septal defect (VSD). Methods. 12 data sets from intraoperative epicardial echocardiographic studies in 5 operations (patient age at operation 3 weeks to 4 years and bodyweight at operation 3.8 to 17.2 kg) after surgical closure of VSD were included in the study. The data sets were analysed as two-dimensional (2D) images on the screen of the ultrasound system as well as holograms in an I-space virtual reality (VR) system. The 2D images were assessed for tricuspid valve function. In the I-Space, a 6 degrees-of-freedom controller was used to create the necessary projectory positions and cutting planes in the hologram. The holograms were used for additional assessment of tricuspid valve leaflet mobility. Results. All data sets could be used for 2D as well as holographic analysis. In all data sets the area of interest could be identified. The 2D analysis showed no tricuspid valve stenosis or regurgitation. Leaflet mobility was considered normal. In the virtual reality of the I-Space, all data sets allowed to assess the tricuspid leaflet level in a single holographic representation. In 3 holograms the septal leaflet showed restricted mobility that was not appreciated in the 2D echocardiogram. In 4 data sets the posterior leaflet and the tricuspid papillary apparatus were not completely included. Conclusion. This report shows that dynamic holographic imaging of intraoperative postoperative echocardiographic data regarding tricuspid valve function after VSD closure is feasible. Holographic analysis allows for additional tricuspid valve leaflet mobility analysis. The large size of the probe, in relation to small size of the patient, may preclude a complete data set. At the moment the requirement of an I-Space VR system limits the applicability in virtual reality 3D echocardiography in clinical practice.

Clinical condition at mid-to-late follow-up after transatrial-transpulmonary repair of tetralogy of Fallot (Article)

2007-02-01T00:00:00Z

Objectives: To assess the clinical condition at mid-to-late follow-up in tetralogy of Fallot corrected by a transatrial-transpulmonary approach at a young age and to identify risk factors associated with right ventricular dilation/dysfunction and with decreased exercise tolerance. Methods: Patients with tetralogy of Fallot underwent cardiac magnetic resonance imaging, maximal bicycle ergometry, electrocardiography, Holter monitoring, and spirometry. Multivariate linear regression analyses were used to determine independent predictors for selected clinical parameters. Results: Fifty-nine patients (mean ± SD), age at repair 0.9 ± 0.5 years, interval since repair 14 ± 5 years, were included. The median pulmonary regurgitant fraction was 32% (0%-57%). Compared with published data on healthy controls, Fallot patients had significantly larger right ventricular end-diastolic and end-systolic volumes and smaller right ventricular and left ventricular ejection fractions. Maximum oxygen consumption was 97% ± 17% and maximum workload 89% ± 13% of predicted. Median QRS duration was 110 ms (82-161 ms). No important ventricular arrhythmias were found. Compared with patients without a transannular patch, patients with a patch had more pulmonary regurgitation, a larger right ventricle, worse right ventricular and left ventricular ejection fractions, but comparable exercise capacity. Multivariate regression analysis identified the following independent determinants for larger right ventricular volumes: longer interval since repair, longer QRS duration, and higher pulmonary regurgitation percentage. The following were independent determinants for smaller right ventricular ejection fraction: abnormal right ventricular outflow tract wall motion, longer interval since repair, and longer QRS duration. For smaller maximum oxygen consumption, the independent determinants were smaller right ventricular ejection fraction and longer QRS duration. Conclusions: At mid-to-late follow-up, clinical condition in tetralogy of Fallot corrected according to contemporary surgical approaches appears well preserved. However, even these patients show right ventricular dilation and dysfunction associated with impaired functional capacity. Abnormalities relate to right ventricular outflow tract motion abnormalities, longer interval since repair, longer QRS duration, and more severe pulmonary regurgitation.

Dynamic 3D echocardiography in virtual reality. (Article)

2005-12-23T00:00:00Z

BACKGROUND: This pilot study was performed to evaluate whether virtual reality is applicable for three-dimensional echocardiography and if three-dimensional echocardiographic 'holograms' have the potential to become a clinically useful tool. METHODS: Three-dimensional echocardiographic data sets from 2 normal subjects and from 4 patients with a mitral valve pathological condition were included in the study. The three-dimensional data sets were acquired with the Philips Sonos 7500 echo-system and transferred to the BARCO (Barco N.V., Kortrijk, Belgium) I-space. Ten independent observers assessed the 6 three-dimensional data sets with and without mitral valve pathology. After 10 minutes' instruction in the I-Space, all of the observers could use the virtual pointer that is necessary to create cut planes in the hologram. RESULTS: The 10 independent observers correctly assessed the normal and pathological mitral valve in the holograms (analysis time approximately 10 minutes). CONCLUSION: this report shows that dynamic holographic imaging of three-dimensional echocardiographic data is feasible. However, the applicability and use-fullness of this technology in clinical practice is still limited.

Decline in ventricular function and clinical condition after Mustard repair for transposition of the great arteries (a prospective study of 22-29 years). (Article)

2004-07-01T00:00:00Z

BACKGROUND: Great concern exists about the ability of the anatomic right ventricle to sustain the systemic circulation in patients with transposition of the great arteries who have undergone a Mustard procedure. A prospective study was made to examine long-term survival, clinical outcome, and right ventricular function 25 years after surgery. METHODS: Ninety-one consecutive patients underwent the Mustard procedure between 1973 and 1980. After 14 years and again after 25 years (range 22-29 years), patients were studied with ECG, echocardiography, exercise testing, and Holter monitoring. RESULTS: The cumulative survival and event-free survival were 77% and 36%, respectively, after 25 years. Reoperation was necessary in 46%. No major loss of sinus rhythm was found. While all patients had good right ventricular function 14 years after repair, 61% of patients showed moderate-to-severe dysfunction after 25 years, when studied by echocardiography. Furthermore, the QRS complex widened and exercise capacity decreased. CONCLUSION: The anatomic right ventricle appears to be unable to sustain the systemic circulation at long-term follow-up and the clinical condition of patients late after Mustard repair is declining. We can expect more deaths or need for heart transplantation in the next decade.

Outcome of patients after surgical closure of ventricular septal defect at young age: longitudinal follow-up of 22-34 years. (Article)

2004-06-01T00:00:00Z

BACKGROUND: Long-term survival and clinical outcome after surgical closure of a VSD is poorly documented. Such data are important for the future perspectives, medical care, employability, and insurability of these patients. METHODS: 176 consecutive patients underwent surgical closure of an isolated VSD between 1968 and 1980 in our hospital. A systematic follow-up study was performed in 1990 and again in 2001. FINDINGS: Late survival was poorer than in the general population. Pulmonary hypertension and right ventricular hypertrophy were present in the 4 patients who died suddenly, late after operation. During follow-up no new pulmonary hypertension became manifest. Re-operations were necessary in 6%. Some patients (4%) developed sinus node disease late after repair, requiring pacemaker implantation. At last follow-up (91 survivors) 92% of the patients were in NYHA class I. Pulmonary hypertension was found in 4%, and aorta insufficiency in 16%. Patients experienced difficulties when applying for insurance. CONCLUSION: Among patients with surgically repaired VSDs, late results were good, although some late sudden deaths occurred in the patients with pulmonary hypertension. Furthermore, some patients developed sinus node disease late after repair, requiring pacemaker implantation. Employability is good, but pregnancy and insurance matters need further attention.

Images in cardiovascular medicine. Multislice computed tomography for the evaluation and follow-up of stenting of aortic coarctation. (Article)

2004-01-01T00:00:00Z

Psychosocial functioning of the adult with congenital heart disease: a 20-33 years follow-up (Article)

2003-04-01T00:00:00Z

AIMS: Since knowledge about the psychosocial function of adult patients with congenital heart disease is limited, we compared biographical characteristics, and emotional and social functioning of these patients with that of the reference groups. METHODS AND RESULTS: Patients with congenital heart disease (N=362, aged 20-46 years), belonging to five diagnostic groups, were subjected to extensive medical and psychological examination, 20-33 years after their first open heart surgery. All the patients were seen by the same psychologist, who examined their psychosocial functioning using a structured interview and questionnaires. The majority (78%)was living independently and showed favourable outcome regarding the marital status. Among married/cohabitant patients, 25-39-year-olds showed normal offspring rates. None of the 20-24-year-old patients had any children. The offspring rate dropped after the age of 40. The proportion of adult patients with a history of special education was high (27%). Accordingly, patients showed lower educational and occupational levels compared to reference groups. As regard to the emotional and social functioning (leisure-time activities), the sample showed favourable results. CONCLUSIONS: Overall, this sample of patients with congenital heart disease seemed capable of leading normal lives and seemed motivated to make good use of their abilities.

Excellent survival and low incidence of arrhythmias, stroke and heart failure long-term after surgical ASD closure at young age. A prospective follow-up study of 21-33 years (Article)

2003-01-01T00:00:00Z

AIMS: Although studies have suggested good long-term results, arrhythmias, pulmonary hypertension and left ventricular dysfunction are mentioned as sequelae long-term after surgical atrial septal defect closure at young age. Most studies were performed only by questionnaire and in a retrospective manner. The long-term outcome is very important with regard to future employment and acceptance on insurance schemes. METHODS AND RESULTS: One hundred and thirty-five consecutive ASD-patients, operated on in childhood, were studied longitudinally with ECG, echocardiography, exercise testing and Holter-recording 15 (10-22) and 26 (21-33) years after surgery. During follow-up no cardiovascular mortality, stroke, heart failure and no pulmonary hypertension occurred. Symptomatic supraventricular tachyarrhythmias were present in 6% after 15 years, and an additional 2% occurred in the last decade; 5% needed pacemaker implantation. No relation was found between arrhythmias and type of ASD, baseline data, right ventricular dimensions, or age at operation. Left and right ventricular function and dimension remained unchanged. Slightly more patients had right atrial dilatation at last follow-up. Exercise capacity was comparable with the normal Dutch population. CONCLUSIONS: The long-term outcome after ASD closure at young age shows excellent survival and low morbidity. The incidence of supraventricular arrhythmias is lower than in natural history studies of ASD patients and also lower than after surgical correction at adult age.

Excellent survival and low incidence of arrhythmias, stroke and heart failure long-term after surgical ASD closure at young age (Article)

2003-01-01T00:00:00Z

Aims Although studies have suggested good long-term results, arrhythmias, pulmonary hypertension and left ventricular dysfunction are mentioned as sequelae long-term after surgical atrial septal defect closure at young age. Most studies were performed only by questionnaire and in a retrospective manner. The long-term outcome is very important with regard to future employment and acceptance on insurance schemes. Methods and Results One hundred and thirty-five consecutive ASD-patients, operated on in childhood, were studied longitudinally with ECG, echocardiography, exercise testing and Holter-recording 15 (10–22) and 26 (21–33) years after surgery. During follow-up no cardiovascular mortality, stroke, heart failure and no pulmonary hypertension occurred. Symptomatic supraventricular tachyarrhythmias were present in 6% after 15 years, and an additional 2% occurred in the last decade; 5% needed pacemaker implantation. No relation was found between arrhythmias and type of ASD, baseline data, right ventricular dimensions, or age at operation. Left and right ventricular function and dimension remained unchanged. Slightly more patients had right atrial dilatation at last follow-up. Exercise capacity was comparable with the normal Dutch population. Conclusions The long-term outcome after ASD closure at young age shows excellent survival and low morbidity. The incidence of supraventricular arrhythmias is lower than in natural history studies of ASD patients and also lower than after surgical correction at adult age.

Aortic valve and aortic arch pathology after coarctation repair (Article)

2003-01-01T00:00:00Z

OBJECTIVE: To investigate the incidence of clinical problems related to a bicuspid valve (aortic stenosis and regurgitation) and the incidence of ascending aorta and aortic arch pathology in combination with coarctation repair. PATIENTS: 124 adult patients after surgical correction of aortic coarctation were studied. The incidence of aortic valve, ascending aorta, and aortic arch pathology was determined using echocardiography and magnetic resonance imaging. The median age at coarctation repair was 9 years and at last follow up 28 years. RESULTS: Three patients died from aorta pathology. Aortic valve disease was found in 63% of the patients, requiring an intervention in 22%, at a median of 13 years after coarctation repair. Ascending aorta dilatation was observed in 28% and aortic arch abnormalities in 23%, among whom kinking of the aortic arch was found in 12%. Antihypertensive medication was used in 24%. In the patients with hypertension the age at operation and age at follow up were significantly higher (p = 0.0001 and p < 0.0001, respectively). CONCLUSION: In addition to the well known problems of hypertension and recoarctation, aortic valve and aortic arch pathology are commonly encountered in patients with previous coarctation repair. Aortic abnormalities may predispose to dilatation and dissection, thus necessitating careful lifelong attention in all patients with coarctation.

Before predicting survival in children with pulmonary hypertension and congenital heart disease... [7] (multiple letters) (Article)

1998-05-25T00:00:00Z

Long-term outcome after surgery for congenital heart disease in infancy and childhood (Doctoral Thesis)

1995-05-24T00:00:00Z

Before the era of cardiac surgery about half of the patients born with a congenital heart defect died within the first year of life. Survival until adulthood was very rare for patients with transposition of the great arteries (20 years survival <1 %), rare with tetralogy of Fallot (20 years survival <10%) and although survival beyond two decades was more common for patients with atrial septal defect, ventricular septal defect and pulmonary stenosis, their life expectancy also was considerably reduced.