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Biomedical subjects

W A Helbing

Publications and source records attributed to W A Helbing.

At least 19 recordsLinked to original sources

Health-related Quality of Life in children and adolescents after invasive treatment for congenital heart disease.

Since the 1980s treatment techniques for congenital heart disease (ConHD) have gradually evolved. Therefore, actual information on the outcomes, including quality of life is required. Health-related quality of life was assessed long-term in four diagnostic groups of children, who underwent invasive treatment for ConHD between 1990 and 1995. The scores on the TNO-AZL Child Quality of Life Questionnaire (TACQOL) of both children with ConHD and their parents were compared with those of a same-aged reference group. The total sample of ConHD children (n = 113, 8-15 years old) obtained significantly lower mean scores on motor functioning, cognitive functioning, and positive emotional functioning than reference peers, reflecting an experience of poorer functioning. ConHD children, aged 8-11 years, obtained lower mean scores on 5 of the 7 TACQOL scales than reference peers. They also had a lower score on positive emotional functioning than 12- to 15-year-old ConHD children. The total sample of ConHD children obtained lower outcomes compared to their parents on 4 of the 7 TACQOL scales. No significant differences were found in health-related quality of life between ConHD boys and girls, neither between different diagnostic groups. Overall, this sample of recently treated ConHD children showed a worse health-related quality of life compared to reference groups. These findings deserve further attention.

Adolescent↗

Maternal hyperhomocysteinaemia is a risk factor for congenital heart disease.

OBJECTIVE: To investigate the inter-relation between mother and infant homocysteine, folate and vitamin B12 status and the risk of a child with congenital heart disease (CHD). DESIGN: Case-control study. SETTING: Erasmus MC, University Medical Centre, Rotterdam, the Netherlands. POPULATION: Participants were 149 case-mothers and their children with CHD (n = 151) and 183 control-mothers with their children (n = 175). METHODS: Approximately 17 months after the index-pregnancy maternal fasting, children's random venous blood samples were drawn to measure plasma total homocysteine, serum and red blood cell (RBC) folate, and serum vitamin B12 concentrations. Data were compared between cases and controls using the Mann-Whitney U test. The biochemical parameters were dichotomised according to the cutoff value of the 10th percentile of vitamin concentrations and the 90th percentile of homocysteine concentrations based on control data. Risk estimates for the association between CHD and the biochemical parameters were estimated in a logistic regression model. MAIN OUTCOME MEASURES: Medians (minimum-maximum) and odds ratios (OR) (95% confidence intervals [CI]). RESULTS: The OR (95% CI) of having a child with CHD was 2.9 (1.4-6.0) for maternal hyperhomocysteinaemia (>14.3 micromol/l). This finding is substantiated by a significant concentration-dependent risk (Ptrend = 0.004). Hyperhomocysteinaemic case-mothers showed significantly lower serum folate and vitamin B12 concentrations than normohomocysteinaemic case-mothers. Serum and RBC folate concentrations were significantly higher in case-children than that in control-children. CONCLUSIONS: Maternal hyperhomocysteinaemia is associated with an increased risk of CHD, partially due to low folate and vitamin B12 status. The folate status of children warrants further investigation.

Adult↗

Cardiovascular response to physical exercise in adult patients after atrial correction for transposition of the great arteries assessed with magnetic resonance imaging.

OBJECTIVE: To assess with magnetic resonance imaging (MRI) cardiovascular function in response to exercise in patients after atrial correction of transposition of the great arteries (TGA). METHODS: Cardiac function at rest and during submaximal exercise was assessed with MRI in 27 patients with TGA (mean (SD) age 26 (5) years) late (23 (2) years) after atrial correction and in 14 control participants (25 (5) years old). RESULTS: At rest, only right ventricular ejection fraction was significantly lower in patients than in controls (56 (7)% v 65 (7)%, p < 0.05). In response to exercise, increases in right ventricular end diastolic (155 (55) ml to 163 (57) ml, p < 0.05) and right ventricular end systolic volumes (70 (34) ml to 75 (36) ml, p < 0.05) were observed in patients. Furthermore, right and left ventricular stroke volumes and ejection fraction did not increase significantly in patients. Changes in right ventricular ejection fraction with exercise correlated with diminished exercise capacity (r = 0.43, p < 0.05). CONCLUSIONS: In patients with atrially corrected TGA, MRI showed an abnormal response to exercise of both systemic right and left ventricles. Exercise MRI provides a tool for close monitoring of cardiovascular function in these patients, who are at risk for late death.

Adult↗

[Good results after closure of a arterial septal defect during heart catheterisation instead of surgery].

OBJECTIVE: Evaluation of the first results in the Netherlands of percutaneous and transvenous closure of an ASD II in children with an Amplatzer Septal Occluder (ASO). DESIGN: Prospective. METHOD: Data were collected from children with an ASD II prior to, during and up to 24 months after the insertion of an ASO during heart catheterisation in Leiden University Hospital, the Netherlands. RESULTS: Between 1 January 1998 and 29 February 2000, 28 patients (12 girls, 16 boys; mean age: 74 months (range: 15-198 months)) underwent heart catheterisation to close an ASD II with an ASO. In 26 patients an ASO could be placed without significant complications. The size of the device varied from 9-34 mm (median 16 mm). In one patient ASD closure was not attempted because of multiple ASDs. In another patient the procedure was stopped after air embolism into the coronary arteries had occurred during preparation of ASO implantation. In 23/26 patients with an implanted ASO, no residual shunt was present after 24 hours. One child, in whom the defect was found to be closed after 24 hours and after three weeks, returned abroad and was lost to follow-up. After one year all defects (n = 22) were completely closed. CONCLUSION: Percutaneous transvenous closure of an ASD II with an ASO was possible, was not associated with any significant complications and had a high success rate, even in relatively young children with large defects.

Adolescent↗

ECG predictors of ventricular arrhythmias and biventricular size and wall mass in tetralogy of Fallot with pulmonary regurgitation.

BACKGROUND: In patients with the tetralogy of Fallot, QRS prolongation predicts malignant ventricular arrhythmias. QRS prolongation may result from right ventricular dilatation. The relation of ECG markers to biventricular wall mass and volumes has not been assessed. OBJECTIVE: To investigate the relations of surface ECG markers of depolarisation and repolarisation to right and left ventricular volume and biventricular wall mass. METHODS: 37 Fallot patients (mean (SD) age 17 (9) years) were studied 14 (8) years after surgical repair; 34 had important pulmonary regurgitation. Left and right ventricular size was assessed from tomographic magnetic resonance imaging (MRI), and the amount of pulmonary regurgitation by velocity mapping MRI. QT, QRS, and JT duration and interlead dispersion markers were derived from a standard 12 lead ECG. RESULTS: Mean QRS duration was significantly prolonged (133 (31) v 91 (11) ms in controls), as were dispersion of QRS (36 (17) v 20 (6) ms), QT interval (87 (48) v 42 (20) ms), and JT interval (93 (48) v 42 (19) ms). Biventricular volumes were increased (right ventricular end diastolic volume, 129 (41) v 70 (9) ml/m(2); left ventricular end diastolic volume, 83 (16) v 69 (10) ml/m(2)), as was right ventricular wall mass (24 (7) v 17 (2) g/m(2)). QRS duration correlated best with right ventricular mass (r = 0.55, p < 0.01). CONCLUSIONS: In patients operated on for tetralogy of Fallot and with pulmonary regurgitation, ECG predictors of ventricular arrhythmias are influenced by several mechanical factors that may occur simultaneously. These include increased right ventricular volume, but also increases in left ventricular volume and in right and left ventricular wall mass.

Adolescent↗

Prolonged cardiac recovery from exercise in asymptomatic adults late after atrial correction of transposition of the great arteries: evaluation with magnetic resonance flow mapping.

After atrial correction of transposition of the great arteries (TGA), dysfunction of the systemic right ventricle at rest and during exercise has been reported. Information on changes in systemic right ventricular function during recovery from exercise is lacking. This study evaluates cardiac recovery from supine exercise using magnetic resonance (MR) imaging in patients with asymptomatic TGA after atrial correction. Flow in the ascending aorta, representing stroke volume of the systemic ventricle, was assessed with MR flow mapping in 10 asymptomatic patients with atrially corrected TGA and in 12 controls at rest during exercise and an 8-minute recovery period. In response to exercise, the patients had a smaller increase in heart rate, stroke volume, and cardiac output than did controls. After exercise, no significant difference in halftime of heart rate recovery was observed (patients, 48 +/- 7 seconds; controls, 39 +/- 4 seconds [p >0.05]). In the patients, the time course of stroke volume recovery was significantly different (p <0.001). Stroke volume in the patients, as a percent difference from rest, remained significantly elevated, from 2.5 minutes (+16 +/- 5% vs +7 +/- 6%; p <0.05) to 8 minutes (+4 +/- 7% vs -3 +/- 5%; p <0.05) after exercise. Subsequently, cardiac output remained significantly elevated, from 4.5 minutes (+27 +/- 13% vs +15 +/- 11%; p <0.05) to 7 minutes (+22 +/- 11% vs +12 +/- 12%; p <0.05) after exercise. We conclude that heart rate recovery is within normal limits in patients with atrially corrected TGA. Furthermore, cardiac recovery from exercise, assessed with MR flow mapping, is prolonged in patients with asymptomatic TGA after atrial correction. Abnormal recovery may reflect dysfunction of the systemic right ventricle and an altered metabolic response to exercise.

Adolescent↗

Biventricular response to supine physical exercise in young adults assessed with ultrafast magnetic resonance imaging.

Simultaneous assessment of left ventricular (LV) and right ventricular (RV) response to exercise is limited with the current imaging modalities. Magnetic resonance imaging (MRI) techniques are now under development that allow near real-time evaluation of biventricular function under physical stress. This approach may open new avenues to study heart function in response to exercise in health and disease. The aim of this study was to evaluate biventricular response to supine physical exercise using ultrafast MRI. Biventricular volumes and function were examined in 16 healthy volunteers (mean age 18 +/- 2 years) using an ultrafast MRI sequence at rest and during an exercise protocol on a MRI compatible bicycle ergometer. Exercise level was individualized at the workload corresponding to 60% of the maximal oxygen uptake. All subjects completed the exercise MRI examination, allowing functional evaluation. Stroke volume of both ventricles increased from rest to exercise (left ventricle, 89 +/- 14 ml vs 102 +/- 19 ml, p < 0.05; right ventricle, 88 +/- 14 ml vs 101 +/- 16 ml, p < 0.05). Ejection fraction also increased in both ventricles from rest to exercise (left ventricle, 63 +/- 6% vs 74 +/- 6%, p < 0.05; right ventricle, 61 +/- 6% vs 70 +/- 6%, p < 0.05). End-systolic volume of the left and right ventricles decreased from rest to exercise (left ventricle, -33 +/- 12%, p < 0.05; right ventricle, -25 +/- 12%, p < 0.05), whereas LV and RV end-diastolic volumes remained unchanged. The results fit well with current concepts of cardiac physiology, and therefore we conclude that ergometer-induced exercise MRI is a valid approach to assess physiologic changes in LV and RV function simultaneously.

Adolescent↗

Biventricular systolic function in young lambs subject to chronic systemic right ventricular pressure overload.

In various clinical situations of congenital heart disease, the right ventricle (RV) is subject to a chronic systemic pressure overload which affects biventricular function and may progress to the development of RV failure. Young lambs (2-3 wk old) underwent adjustable pulmonary artery banding (PAB) at systemic (aortic) level for 8 wk. Biventricular function was determined by using load-independent indexes of global ventricular contractile performance by the end-systolic pressure-volume relationship (ESPVR) using the conductance catheter at baseline and during dobutamine infusion. PAB resulted in a significant fivefold increase in RV end-systolic pressure (12-64 mmHg) and a doubling of the RV-to-left ventricular (LV) wall thickness ratio (P < 0.01). RV global contractile performance increased significantly, as indicated by an increased slope of the ESPVR. Compared with age-matched control lambs, cardiac output decreased from 2.6 to 1.6 l/min (P < 0.05) whereas heart rates were equal. In contrast with RV volume, LV volume decreased significantly after PAB (P < 0.01), whereas the LV-ESPVR slope was unchanged. In the PAB group, the RV, but not the LV, showed a reduced response to dobutamine. We concluded that chronic RV pressure overload for 8 wk results in diminished pump function despite compensatory increased RV global contractile performance.

Age Factors↗

Spin echo MRI in the evaluation of hearts with a double outlet right ventricle: usefulness and limitations.

The surgical approach to a double outlet right ventricle (DORV) is dependent on the spatial relationship of the semilunar valves, outflow tracts and ventricular septal defect (VSD). The purpose of the study was to assess the value of MRI for the evaluation of cardiovascular anatomy in patients before and after surgery for DORV. Spin echo MRI was performed in 12 patients with DORV (eight preoperative and four postoperative patients). Thin-section MRI was performed in three orthogonal planes and selected angulated views were obtained. Conventional imaging by color Doppler echocardiography and cine-angiocardiography and surgical findings, when present, served as the reference standards. The results found that the spatial relationship between semilunar valves and VSD was accurately assessed by MRI in eight out of eight preoperative patients. In the four postoperative cases MRI depicted the morphology of both outflow tracts and provided adequate information on their patency. Of the eight preoperative patients, five have undergone corrective surgery and the MRI findings were confirmed. MRI provided additional information to conventional imaging preoperatively in three cases in which the VSD opened into the outlet portion of the DORV, without there being a direct relation to a semilunar valve. In two preoperative cases in which the VSD was directly committed to the aorta, conventional imaging was conclusive. MRI was unable to depict aberrant chordae tendineae in four out of four cases. We conclude that MRI provides accurate additional anatomic information in patients with DORV, which is helpful in presurgical planning as well as during follow-up. Spin echo MRI does not visualize aberrant chordae tendineae.

Adolescent↗

Postoperative evaluation of congenital heart disease by magnetic resonance imaging.

In the last four decades the survival of patients with corrected or palliated congenital heart disease has increased dramatically. However, post-operative abnormalities frequently occur and therefore a noninvasive imaging tool is mandatory for the timely detection of morphological as well as functional abnormalities. Magnetic resonance imaging (MRI) is ideally suited for the noninvasive diagnosis and post-operative follow-up of congenital heart disease. Spin-echo MRI is able to visualize structures that may be difficult to assess with other noninvasive image modalities and is sensitive in the detection of post-interventional stenoses or aneurysms. Because the function of the ventricles may deteriorate over time after correction or palliation of a congenital cardiac malformation, the use of gradient-echo MRI is essential in the follow-up after correction or palliation, as no other conventional technique allows such detailed evaluation of ventricular function, without geometrical assumptions. Phase-contrast MRI is well suited to assess valvular function, allowing accurate measurement of regurgitation or stenosis. Shunt quantification is another application of phase-contrast MRI. J. Magn. Reson. Imaging 1999;10:656-666.

Follow-Up Studies↗

Double outlet right ventricle assessed with magnetic resonance imaging.

In this article the value of magnetic resonance (MR) imaging for the evaluation of double outlet right ventricle (DORV) is reviewed from the literature and illustrated with several cases. MR imaging can be used for the determination of cardiac anatomy at initial diagnosis and may provide functional information during the follow-up of patients after surgical correction.

Child, Preschool↗

Left ventricular function in adults with mild pulmonary insufficiency late after Fallot repair.

OBJECTIVE: To assess left ventricular function in adult Fallot patients with residual pulmonary regurgitation. SETTING: The radiology department of a tertiary referral centre. PATIENTS: 14 patients with chronic pulmonary regurgitation and right ventricular volume overload after repair of tetralogy of Fallot and 10 healthy subjects were studied using magnetic resonance imaging. MAIN OUTCOME MEASURES: Biventricular volumes, global biventricular function, and regional left ventricular function were assessed in all subjects. RESULTS: The amount of pulmonary regurgitation in patients (mean (SD)) was 25 (18)% of forward flow and correlated significantly with right ventricular enlargement (p < 0.05). Left ventricular end diastolic volume was decreased in patients (78 (11) v 88 (10) ml/m(2); p < 0.05), ejection fraction was not significantly altered (59 (5)% v 55 (7)%; NS). No significant correlation was found between pulmonary regurgitation and left ventricular function. Overall left ventricular end diastolic wall thickness was significantly lower in patients (5.06 (0.72) v 6.06 (1.06) mm; p < 0. 05), predominantly in the free wall. At the apical level, left ventricular systolic wall thickening was 20% higher in Fallot patients (p < 0.05). Left ventricular shape was normal. CONCLUSIONS: Adult Fallot patients with mild chronic pulmonary regurgitation and subsequent right ventricular enlargement showed a normal left ventricular shape and global function. Although the left ventricular free wall had reduced wall thickness, compensatory hypercontractility of the apex may contribute to preserved global function.

Adult↗

Quantification of pulmonary and systemic blood flow by magnetic resonance velocity mapping in the assessment of atrial-level shunts.

The objective of this study was to asses the feasibility and accuracy of magnetic resonance (MR) velocity mapping to calculate pulmonary-to-systemic flow ratio (Qp:Qs) in patients with a suspected or diagnosed atrial-level shunt. During a one-year period, all patients referred to our department for further evaluation of an atrial-level shunt underwent the same imaging protocol. Multiphase-multisection gradient-echo MR image sets of the heart were acquired to measure left and right ventricular stroke volumes for validation. Ascending aorta and main pulmonary artery volume flow were measured with MR velocity mapping. Qp:Qs ratios were calculated from both stroke volume data and flow data. Twelve patients, including 6 children, were studied. Six patients had an established diagnosis of atrial septal defect, and the other 6 patients were suspected to have an atrial-level shunt. Measurements of left and right ventricular stroke corresponded closely with those of aortic (r = 0.98) and pulmonary flow (r = 0.99) respectively, and Qp:Qs flow ratios agreed with stroke volume ratios (r = 0.92). In 5 patients with a suspected shunt, the diagnosis could be rejected. Shunts were demonstrated in the other 7 patients. M(r) velocity mapping offers an accurate method to measure aortic and pulmonary artery volume flow that can be useful in the evaluation of atrial-level shunts, in order to establish a definite diagnosis and/or to quantify the Qp:Qs ratio.

Adolescent↗

Right ventricular diastolic function in children with pulmonary regurgitation after repair of tetralogy of Fallot: volumetric evaluation by magnetic resonance velocity mapping.

OBJECTIVES: We sought to assess right ventricular diastolic function in young patients with corrected tetralogy of Fallot and pulmonary regurgitation. BACKGROUND: Pulmonary regurgitation is an important problem in repair of tetralogy of Fallot. Its effects on right ventricular diastolic function in children are unknown. METHODS: Nineteen children with repair of tetralogy of Fallot (mean age [+/- SD] 12 +/- 3 years, mean age at operation 1.5 +/- 1) and 12 healthy children were studied. Summation of magnetic resonance velocity mapping pulmonary and tricuspid volume flow curves provided right ventricular time-volume curves. Ventricular size was assessed with tomographic magnetic resonance imaging (MRI). Graded exercise testing was performed. RESULTS: Systematic and random differences (mean +/- SD) of velocity mapping and Doppler tricuspid time to peak velocities (peak E: 1 +/- 26 ms, r = 0.43; peak A: 2 +/- 11 ms, r = 0.76), E/A ratio (0.04 +/- 0.5, r = 0.63) and duration of pulmonary regurgitation (20 +/- 35 ms, r = 0.74) were satisfactory. In 6 patients (group I), late diastolic forward pulmonary artery flow was absent; in 13 patients (group II), this flow contributed 1% to 14% to right ventricular stroke volume. Significant differences were increased deceleration time (315 +/- 91 vs. 168 +/- 28 ms, p < 0.001), decreased filling fraction (44 +/- 11 vs. 55 +/- 16%, p = 0.02) and increased peak early filling rate (378 +/- 124 vs. 286 +/- 112 ml/s, p = 0.018) between control subjects and group I, and increased deceleration time (230 +/- 40, p = 0.03) between control subjects and group II. Pulmonary regurgitation, ventricular size and ejection fraction did not differ significantly between patient groups. Exercise function was diminished with restrictive right ventricular physiology (p < 0.001, group II vs. control subjects). CONCLUSIONS: Impaired relaxation and restriction to filling affect right ventricular function in children with repair of tetralogy of Fallot and pulmonary regurgitation. Restrictive right ventricular physiology is associated with decreased exercise function.

Adolescent↗

Cine gradient-echo MR imaging and MR velocity mapping in the evaluation of congenital heart disease.

Cine gradient-echo (GRE) magnetic resonance (MR) imaging and MR velocity mapping can be applied to assess cardiovascular function and to measure volumetric flow in patients with repaired and untreated congenital heart disease. GRE images, particularly when displayed in a cine loop, are very useful for visualizing surgical anastomoses and conduits and for detecting stenosis, regurgitation, and left-to-right shunts. Multisection cine GRE imaging is a suitable method for measuring global right ventricular function, an important issue in congenital heart disease. MR velocity mapping is used for quantitative assessment of hemodynamics. Pressure gradients can be estimated from peak blood flow velocities in stenotic pulmonary arteries or surgical conduits. Because flow volume can be measured with MR velocity mapping, main-, left-, and right pulmonary artery flow, valvular regurgitation, left-to-right shunts, and ventricular filling can all be quantified. The abilities to elucidate complex cardiac anatomy and to measure cardiac function and flow in one examination make MR imaging a useful comprehensive tool for follow-up of congenital heart disease.

Adult↗

Biventricular systolic function and mass studied with MR imaging in children with pulmonary regurgitation after repair for tetralogy of Fallot.

PURPOSE: To study biventricular systolic function and mass of the heart in young patients with residual pulmonary regurgitation who had undergone surgical correction of tetralogy of Fallot. MATERIALS AND METHODS: Transverse gradient-echo magnetic resonance (MR) images covering both ventricles were obtained in 19 patients who had undergone corrective surgery for tetralogy of Fallot at the age of 1.5 years +/- 1 and in 12 age-matched control subjects. In addition, MR velocity maps of the pulmonary artery were obtained. Biventricular volumes, ejection fraction and myocardial mass, and pulmonary flow volumes were measured. Exercise tests were performed in 17 patients. RESULTS: The right ventricular ejection fraction was lower (P < .001) and the right ventricular mass was higher (P < .0005) in patients than in control subjects; the left ventricular ejection fraction was lower (P < .0005) in patients and correlated statistically significantly with pulmonary regurgitation (r = -.68; P < .005). Exercise performance inversely correlated with pulmonary regurgitation (tau = -0.5; P = .01). CONCLUSION: In children who undergo early surgical repair of tetralogy of Fallot, residual pulmonary regurgitation correlates with biventricular systolic dysfunction and diminished exercise capacity. Despite successful surgical correction, right ventricular hypertrophy may persist.

Case-Control Studies↗