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Biomedical subjects

Barbara J M Mulder

Publications and source records attributed to Barbara J M Mulder.

At least 19 recordsLinked to original sources
PubMed2006

Psychological and cognitive functioning in children and adolescents with congenital heart disease: a meta-analysis.

OBJECTIVE: Findings in the literature are inconsistent on the impact of congenital heart disease (CHD) on the psychological and cognitive functioning of children and adolescents. The aim of the present study was to systematically review this empirical body of literature. METHODS: We conducted a meta-analysis to review studies on behavior problems and cognitive functioning in CHD. RESULTS: Only older children and adolescents with CHD displayed an increased risk of overall, internalizing, and to a lesser extent externalizing behavior problems. In addition, patients with severe CHD exhibited lower cognitive functioning than patients with less severe CHD, specifically with respect to performance intelligence. Moreover, decreased cognitive functioning remained relatively stable across different age groups. CONCLUSIONS: Children with severe heart disease may benefit from interventions specifically targeting perceptual organizational abilities, such as visual-spatial abilities. Moreover, older children and adolescents with CHD may benefit from psychological interventions reducing anxiety symptoms and depression.

Adolescent↗
PubMed2006

Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease.

AIM: To investigate the role of pulmonary arterial hypertension (PAH) in adult patients born with a cardiac septal defect, by assessing its prevalence and its relation with patient characteristics and outcome. METHODS AND RESULTS: From the database of the Euro Heart Survey on adult congenital heart disease (a retrospective cohort study with a 5-year follow-up), the relevant data on all 1877 patients with an atrial septal defect (ASD), a ventricular septal defect (VSD), or a cyanotic defect were analysed. Most patients (83%) attended a specialised centre. There were 896 patients with an ASD (377 closed, 504 open without and 15 with Eisenmenger's syndrome), 710 with a VSD (275, 352 and 83, respectively), 133 with Eisenmenger's syndrome owing to another defect and 138 remaining patients with cyanosis. PAH was present in 531 (28%) patients, or in 34% of patients with an open ASD and 28% of patients with an open VSD, and 12% and 13% of patients with a closed defect, respectively. Mortality was highest in patients with Eisenmenger's syndrome (20.6%). In case of an open defect, PAH entailed an eightfold increased probability of functional limitations (New York Heart Association class >1), with a further sixfold increase when Eisenmenger's syndrome was present. Also, in patients with persisting PAH despite defect closure, functional limitations were more common. In patients with ASD, the prevalence of right ventricular dysfunction increased with systolic pulmonary artery pressure (OR = 1.073 per mm Hg; p<0.001). Major bleeding events were more prevalent in patients with cyanosis with than without Eisenmenger's syndrome (17% vs 3%; p<0.001). CONCLUSION: In this selected population of adults with congenital heart disease, PAH was common and predisposed to more symptoms and further clinical deterioration, even among patients with previous defect closure and patients who had not developed Eisenmenger's physiology.

Adult↗
PubMed2006

Long-term effect of pulmonary valve replacement on QRS duration in patients with corrected tetralogy of Fallot.

OBJECTIVE: To analyse the long-term course of QRS duration after pulmonary valve replacement in patients with a previous correction for tetralogy of Fallot. SETTING: Tertiary referral centres. METHODS: In a retrospective study, 99 adult patients with tetralogy of Fallot, who had undergone a first pulmonary valve replacement late after initial total correction, were identified from the CONCOR (CONgenital CORvitia) registry. Computer-generated QRS durations were obtained from 12-lead electrocardiogram ECG reports in the medical records. A mixed linear regression model was used to analyse the course of QRS duration over time and to identify risk factors for increase in QRS duration over time. Composite end point was created from sudden cardiac death, ventricular tachycardia or implantable cardioverter-defibrillator discharge. RESULTS: In total, 99 patients (57% men, mean (SD) age at pulmonary valve replacement 29 (11) years) with a median follow-up of 4.9 (0.1-16) years were analysed. In patients with preoperative QRS <120 ms, surgery caused no significant change in QRS duration (increase 1.3 (7.9) ms; p = 0.65), and after surgery, QRS duration remained stable over time (increase 0.0064 (0.059) ms/year; p = 0.98). By contrast, in patients with a preoperative QRS of 150-180 ms or QRS > or =180 ms, surgery resulted in QRS shortening (mean decrease 9.9 (SE 4.3) ms, p = 0.021, and 12.2 (SE 2) ms; p<0.001, respectively). During follow-up, a QRS widening 1.1(1.3) ms/year (p<0.001) in both groups was observed. In patients with a preoperative QRS > or =180 ms, no significant difference was observed in the number of patients reaching the composite end point compared with patients with a preoperative QRS of 150-180 ms (25% vs 7%; p = 0.08). However, the former more often reached QRS > or =180 ms again after surgery compared with the latter (53% vs 13%; p = 0.02, respectively). None of the patients with a preoperative QRS > or =180 ms died during follow-up. CONCLUSION: In our study, we observed a decrease in QRS duration directly after surgery, followed by a steady increase, in patients with a preoperative QRS >150 ms. The beneficial effect of pulmonary valve replacement on QRS duration was transient. The risk of developing ventricular arrhythmias after surgery was substantial when preoperative QRS was > or =180 ms, but mortality remained low.

Death, Sudden, Cardiac↗
PubMed2006

Outcome of implantable cardioverter defibrillators in adults with congenital heart disease: a multi-centre study.

AIMS: To investigate outcome and complications of implantable cardioverter defibrillators (ICDs) in adults with congenital heart disease (CHD) and to identify predictors of (in-) appropriate shocks. METHODS AND RESULTS: Sixty-four CHD patients >/= 18 years at first ICD implantation [63% tetralogy of Fallot (TOF) and age at implantation 37 +/- 13 years] were identified using the Dutch adult CHD registry and a Belgian tertiary care centre database. Median follow-up duration was 3.7 years. Early complications included pocket haematoma (n = 3), lead failure (n = 2), and pneumothorax (n = 2). Late complications occurred in 11 (17%) patients, including lead failure (n = 6) and and electrical storm (n = 3). Overall, 30 device-related re-interventions were performed in 20 patients (31%), including four premature generator changes and seven lead replacements. Half of the patients received one or more shocks, and 46 shocks in 15 patients (23%) were classified as appropriate. One hundred and sixty shocks in 26 patients (41%) were classified as inappropriate. No predictors of (in-)appropriate shocks were identified, except TOF being associated with less appropriate shocks than patients with other CHD (HR 0.29, P = 0.02). CONCLUSION: The ICD provided effective therapy in a quarter of adults with CHD with low complication rates. The incidence of inappropriate shocks, however, appeared to be excessive and warrants further attention.

Adult↗
PubMed2006

Cardiovascular magnetic resonance in a pregnant patient with absent pulmonary valve syndrome.

We present a 22 year old Moroccan woman with chronic severe pulmonary regurgitation, who becomes symptomatic in her fourth month of pregnancy. Cardiovascular magnetic resonance, during pregnancy, revealed a large pulmonary aneurysm and turbulent blood flow in the pulmonary trunk with severe pulmonary regurgitation. After gestation, the branch pulmonary arteries were assessed with magnetic resonance angiography and the severely dilated pulmonary arterial trunk and valve were replaced by a pulmonary homograft. We briefly review this rare syndrome, the management of pulmonary regurgitation during pregnancy and the role of CMR during pregnancy.

Adult↗
PubMed2006

Stress-induced heart symptoms and perceptual biases in patients with congenital heart disease.

BACKGROUND: The aim of the present study is to clarify whether biased symptom perception towards heart symptoms may explain a reduced quality of life in patients with congenital heart disease (ConHD). The present study tested the hypothesis that the combination of ConHD and high trait anxiety increases the perception of heart symptoms during acute stress. METHODS: 25 patients and 24 healthy participants completed a stressful computer task. Participant's heart and non-heart symptoms were measured after stress and after relaxation. Heart rate, blood pressure, respiratory rate, and arterial partial pressure of CO2 were monitored continuously. RESULTS: In line with the prediction, a combination of high trait anxiety and ConHD resulted in an increased perception of specifically heart symptoms during stress. Moreover, the increased perception of heart symptoms could not be explained by acute heart dysfunction. CONCLUSIONS: Heart dysfunction is not the only cause of an increased perception of heart symptoms. A history of disease experience in combination with high trait anxiety may increase the perception of heart symptoms during stress and may eventually result in an increased risk of developing a reduced quality of life.

Analysis of Variance↗
PubMed2006

Combined utility of brain natriuretic peptide and cardiac troponin T may improve rapid triage and risk stratification in normotensive patients with pulmonary embolism.

BACKGROUND: Evaluation of the combined utility of brain natriuretic peptide (BNP) and cardiac troponin T (cTnT) for risk stratification in normotensive patients with acute pulmonary embolism. The changing patterns of BNP and cTnT levels during the initial management of pulmonary embolism and their correlation with clinical outcome were the focus of this study. METHODS: BNP and cTnT levels were measured in 28 consecutive normotensive on admission patients (age 53+/-18 years) with acute pulmonary embolism. Twenty-seven healthy age-matched volunteers served as controls (age 42+/-12 years). Blood samples from all patients were obtained during the first hour of presentation. Follow-up blood samples were acquired after 90 days. RESULTS: Six patients (2%) had increased BNP (59+/-30 pmol/L) and cTnT (0.044+/-0.025 ng/mL) and RV dysfunction as diagnosed by echocardiography. Two of these patients died during follow-up as a consequence of RV failure due to pulmonary embolism. Eight patients (29%) had increased BNP (40.6+/-32.6 pmol/L) and normal cTnT at presentation. In four of these patients BNP levels remained increased after treatment (46.9+/-21.0 pmol/L). During follow-up these patients were diagnosed with chronic PE and RV pressure overload (RVSP>40 mm Hg). Fourteen patients (50%) with confirmed pulmonary embolism had normal BNP and cTnT levels at presentation, which remained normal during follow-up. These 14 patients were in good clinical condition during follow-up. CONCLUSIONS: Combined use of BNP and cTnT may be useful in risk stratification of normotensive patients with acute pulmonary embolism. Patients with increased BNP and cTnT are at risk for adverse outcome. Future studies in larger numbers of patients are needed to confirm the usefulness of biomarkers in the clinical management of individual patients.

Adult↗
PubMed2006

Fertility, pregnancy, and delivery after biventricular repair for pulmonary atresia with an intact ventricular septum.

The objective of the present study to investigate fertility, pregnancy, and delivery in women with biventricular repair for pulmonary atresia with an intact ventricular septum (PAIVS). Using a nationwide registry (CONCOR), 37 patients with pulmonary atresia were identified, 6 of whom (aged 21 to 34 years) had biventricular repair for PAIVS. Three PAIVS patients had a total of 5 pregnancies, including 1 abortion and 1 ectopic pregnancy. Besides minor noncardiac complications, the 3 live-birth pregnancies were successful. None of the women reported infertility or irregularities (hypermenorrhea, dysmenorrhea, polymenorrhoea, oligomenorrhea, or amenorrhea) of their natural menstrual cycle. In conclusion, successful pregnancy in women with biventricular repair for PAIVS is possible, and only minor complications were observed. Infertility and menstrual cycle disorders do not appear to be more prevalent than usual.

Adult↗
PubMed2006

Pregnancy after biventricular repair for pulmonary atresia with ventricular septal defect.

Information on pregnancy and delivery in women with biventricular repair for isolated noncomplex pulmonary atresia with a ventricular septal defect (PAVSD) is limited. Using a nationwide congenital heart disease registry (CONgenital CORvitia [CONCOR]), 9 women with biventricular repair for PAVSD (aged 21 to 38 years) were identified. Ten pregnancies were observed in 5 different women with PAVSD, including 3 spontaneous miscarriages. Clinically significant (non)cardiac complications were documented in 3 of 7 completed pregnancies. These complications were: (1) atrioventricular reentry tachycardia with symptomatic right-sided heart failure; (2) eclampsia with hemolysis, elevated liver enzymes, and low platelets syndrome further complicated by abruptio placentae leading to premature delivery of a small-for-gestational-age child; and (3) premature delivery due to cervical insufficiency with antepartum demise of an immature child. Furthermore, none of the women reported infertility. Moreover, none of the women reported irregularities of their natural menstrual cycle (age at menarche 13 years; cycle duration 28 days), with the exception of delayed menarche (>16 years) in 2 patients. In conclusion, successful pregnancy in patients with biventricular repair of PAVSD is possible, although often complicated by serious clinically significant events. Infertility and menstrual cycle disorders do not appear to be more prevalent, except for a high incidence of primary amenorrhea.

Adult↗
PubMed2006

Long-term follow-up of homograft function after pulmonary valve replacement in patients with tetralogy of Fallot.

AIMS: To analyse the long-term outcomes after pulmonary valve replacement (PVR) in patients with a previous correction for tetralogy of Fallot. METHODS AND RESULTS: In a retrospective study, 158 adult patients with a diagnosis of tetralogy of Fallot, who had undergone a PVR after initial total correction in childhood, were identified from the CONCOR (CONgenital CORvitia) registry. All patients underwent 175 PVRs between June 1986 and June 2005. To analyse the predictors for homograft dysfunction and adverse events (death, reoperations, balloon angioplasty), Cox-regression analysis was performed. Overall freedom from significant homograft dysfunction was 66% after 5 years and 47% after 10 years. We could not identify predictors for combined homograft dysfunction. Event-free survival was 78% at 10 years and 68% at 15 years after PVR. Both early significant pulmonary regurgitation (PR) (HR 6.8, P = 0.017) and pulmonary stenosis (PS) (HR 4.0, P = 0.037) after surgery were associated with adverse events. When analysing direct post-operative PR or PS, we observed that in patients with severe, pre-operative PR, right ventricular aneurysm/patch resection resulted in a lower post-operative PR (mean difference grade 0.38 +/- 0.14, P = 0.01). Less significant post-operative PS was associated with a higher diameter of the homograft (HR 0.37, P = 0.006). CONCLUSION: While 47% of the patients in our study were free from homograft dysfunction at 10 years after PVR, event-free survival after PVR remained fairly good (78%). Significant residual lesions directly after surgery influenced event-free survival. A smaller diameter of the pulmonary homograft and severe pre-surgical PR were related to early homograft dysfunction after surgery.

Adult↗
PubMed2006

Delivery of care for adult patients with congenital heart disease in Europe: results from the Euro Heart Survey.

AIMS: The increasing number of adults with congenital heart disease (CHD) has prompted the development of recommendations for the management of these patients and for the organization of their healthcare. The aim of this report is to describe the delivery of care in Europe for adults with congenital cardiac anomalies. METHODS AND RESULTS: As part of the Euro Heart Survey on Adult Congenital Heart Disease, we obtained data from 71 voluntarily participating centres that detailed their care practices for these patients. Forty-eight of these centres were specialist centres and 23 were non-specialist centres. We found that only 19% of the specialist centres complied with defined standards for optimal care structure. The criteria that appeared to be most difficult for all centres to achieve were performing 50 congenital heart operations or more per year and involving nurse specialists in the care of these patients. CONCLUSION: This survey indicated that the provision of care in Europe for adults with congenital heart defects is suboptimal. To fully realize the benefits of cardiac surgery performed in infants and children, continuous effort must be applied by healthcare professionals in order to implement the recommendations on the organization of care for these patients.

Adult↗
PubMed2006

Effects of volume and/or pressure overload secondary to congenital heart disease (tetralogy of fallot or pulmonary stenosis) on right ventricular function using cardiovascular magnetic resonance and B-type natriuretic peptide levels.

The aim of this study was to analyze the effect of pressure and/or volume overload on right ventricular (RV) function and brain natriuretic peptide (BNP) levels in patients with surgically corrected congenital heart disease. Forty-two consecutive patients aged 17 to 57 years (median 30) with congenital heart disease (32 with tetralogy of Fallot and 10 with pulmonary stenosis) were examined. The RV systolic pressure was estimated using Doppler echocardiography. Cardiovascular magnetic resonance imaging was used to obtain the RV volumes, ejection fraction (EF) and corrected EF (cEF). Plasma BNP levels were determined by immunoradiometric assay. Patients were categorized as having volume overload when pulmonary regurgitation was > or =10% and pressure overload when the RV systolic pressure was >40 mm Hg. Patients with RV volume overload had a lower RVEF compared with patients with pressure overload (p = 0.02) and lower left ventricular EF (p <0.001). BNP was higher in patients with volume overload than in patients with pressure overload (p = 0.002). BNP correlated with pulmonary regurgitation, RVEF, RV cEF, and left ventricular EF. In linear regression analysis, RV cEF was an independent predictor for BNP, after adjustment for age. Without the parameter of RV cEF in the regression model, pulmonary regurgitation and RVEF were independently associated with BNP level, after adjustment for age. In conclusion, patients with RV volume overload had higher BNP levels and lower RV function than patients with RV pressure overload. BNP levels were independently associated with the degree of RV volume overload and RV function.

Adolescent↗
PubMed2006

Increased carotid and femoral intima-media thickness in patients after repair of aortic coarctation: influence of early repair.

BACKGROUND: In patients, after repair of aortic coarctation, abnormal function of the proximal precoarctation arterial conduits has been demonstrated, but data on arterial wall structure of proximal and distal arteries in patients are scarce. The aim of our study is to compare intima-media thickness (IMT) of the carotid and femoral arteries in controls and patients after coarctation repair and to identify independent predictors of carotid and femoral IMT. METHODS: A total of 137 patients after repair of aortic coarctation (89 male, median age 29.7 years, range 17-74 years, and median age at repair 6.3 years, range 0.01-46 years) and 46 age-matched controls underwent ambulatory blood pressure monitoring and B-mode ultrasound of the carotid and femoral arteries. RESULTS: Twenty (15%) of the 137 patients were operated on before the age of 1 year ("early repair"). The patients with an early repair were younger compared to the controls and compared with the patients with a late repair (24.5 [10.5] vs 31.6 [8.4] years, P = .005, and vs 32.1 [10.5] years, P < .001, respectively). Eighty patients (59%) had hypertension on the basis of the results of the ambulatory blood pressure monitoring (mean daytime blood pressure > or = 135/85 mm Hg). The prevalence of hypertension was not significantly different between patients with an early or late repair. Although patients with an early repair were younger, both carotid and femoral IMT were increased in these patients compared to controls (common carotid artery 0.57 [0.11] vs 0.49 [0.07] mm, P < .001; common femoral artery 0.48 [0.10] vs 0.44 [0.05] mm, P = .033). In multivariable regression analyses, age at repair was only an independent predictor of femoral and not of carotid IMT. CONCLUSIONS: Patients, after repair of aortic coarctation, have increased carotid and femoral IMT despite normal ambulatory blood pressures and successful previous repair. Early repair seems to preserve postcoarctation arterial wall structure, but seems to have only limited effect on precoarctation vessel walls, which supports the theory of a developmental defect of the proximal aorta and its branches.

Adolescent↗
PubMed2006

Cardiovascular magnetic resonance in the follow-up of patients with corrected tetralogy of Fallot: a review.

Cardiovascular magnetic resonance (CMR) is becoming an important tool in the clinical management of patients with congenital heart disease. Because of the diverse problems patients may face after initial correction for tetralogy of Fallot and the large amount of CMR techniques that can be applied, creating a patient-orientated imaging protocol is a difficult issue. Although it is still not certain what the impact of some parameters, provided by CMR, should be on clinical decision making, new techniques are being developed and applied. In this report, we review the current clinical issues in patients with tetralogy of Fallot and review the current implication and limitations of CMR in this patient category.

Coronary Aneurysm↗
PubMed2005

Obstetric complications in Marfan syndrome.

OBJECTIVE: To investigate the obstetric maternal and neonatal outcome of pregnancy in women with Marfan syndrome. METHODS: Retrospective observational multi-center study based on congenital heart disease registry. RESULTS: Sixty-three of the 122 enrolled women with Marfan syndrome had been pregnant 142 times (including 111 pregnancies>20 weeks, 28 (20%) miscarriages and 3 elective abortions). In 40% of all completed pregnancies an obstetric and/ or neonatal complication occurred. The most important complications were an increased percentage of premature deliveries (n=17, 15%) mainly due to preterm premature rupture of membranes and cervical incompetence and a markedly increased combined fetal and neonatal mortality of 7.1%. An obstetric and/or neonatal complication occurred in a similar proportion of pregnancies in women with a diagnosis of Marfan syndrome before pregnancy versus women with a diagnosis of Marfan syndrome after pregnancy (39% vs. 41%, p=0.85, respectively). CONCLUSION: In addition to cardiovascular complications, pregnancy in women with Marfan syndrome is associated with a high rate of premature deliveries, preterm premature rupture of membranes and increased mortality in the offspring.

Abortion, Spontaneous↗
PubMed2005

Aortic root growth in men and women with the Marfan's syndrome.

The leading cause of premature death in patients with Marfan's syndrome (MS) is type A aortic dissection or rupture due to progressive aortic root dilation. The aim of this study was to analyze aortic root growth in 113 men and 108 women with MS. All patients were prospectively followed with serial echocardiograms of the native aortic root. At baseline, women had on average a 5-mm smaller aortic root diameter adjusted for age than men. Average aortic root growth was 0.42 mm/year (SE 0.05) in men and 0.38 mm/year (SE 0.04) in women. On the basis of aortic root growth rates, the men and women could be divided into 2 normally distributed subgroups: fast and slow growers. Approximately 1 in 7 men (1.5 mm/year, SE 0.5) and approximately 1 in 9 women (1.8 mm/year, SE 0.3) had fast-growing aortic root diameters. Significantly more type A dissections (25% vs 4%, p <0.001) were observed in fast growers than in slow growers; this was found in men and women. Type A dissections were observed in 4 men and 9 women. By reducing the cut-off value by 5 mm for elective aortic root replacement in women, type A dissections could have been prevented in 3 women. In conclusion, guidelines should take gender differences into account, and therefore, the investigators propose reducing the threshold for elective aortic root replacement in women with MS by 5 mm.

Adolescent↗
PubMed2005

Risk of complications during pregnancy after Senning or Mustard (atrial) repair of complete transposition of the great arteries.

AIMS: To investigate magnitude and determinants of risks during pregnancy in women with Mustard or Senning repair for complete transposition of the great arteries (TGA). METHODS AND RESULTS: Using a nationwide registry (CONCOR), 70 women with Senning (23%) or Mustard (77%) repair for TGA were enrolled. A total of 28 patients had 69 pregnancies (two twins), including 17 spontaneous miscarriages and three elective abortions. During 39 of the 49 completed pregnancies, complications were observed. The most important cardiac complication was clinically significant arrhythmia (n=11, 22%), especially occurring in patients with a prior history of arrhythmia. Important general pregnancy complications were preeclampsia (n=5, 10.2%) and pregnancy-induced hypertension (n=4, 8.2%). Obstetric complications included premature rupture of membranes (n=7, 14.3%), premature labour (n=12, 24.4%), premature delivery (n=16, 31.4%), and thrombo-embolic complications (n=2, 4.1%). Mean (singleton) pregnancy duration was 36+/-5 weeks. Eleven of the 51 children (21.6%) were small for gestational age. Foetal and neonatal mortality combined was 11.8% (n=6). No recurrence of congenital heart disease in the offspring was documented. CONCLUSION: In this largest report on pregnancy in women with atrial-corrected TGA to date, a high incidence of obstetric complications and mortality in the offspring was observed.

Abortion, Spontaneous↗