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Biomedical subjects

Jaap Ottenkamp

Publications and source records attributed to Jaap Ottenkamp.

At least 19 recordsLinked to original sources
PubMed2006

Persistent ductus arteriosus in the Brown-Norway inbred rat strain.

Persistent ductus arteriosus (PDA) is a common cardiovascular anomaly in children caused by the pathologic persistence of the left sixth pharyngeal arch artery. The inbred Brown-Norway (BN) rat presents with increased vascular fragility due to an aortic elastin deficit resulting from decreased elastin synthesis. The strikingly high prevalence of PDA in BN rats in a pilot study led us to investigate this vascular anomaly in 12 adolescent BN rats. In all BN rats, a PDA was observed macroscopically, whereas a ligamentum arteriosum was found in adult controls. The macroscopic appearance of the PDA was tubular (n = 2), stenotic (n = 8), or diverticular (n = 2). The PDA had the structure of a muscular artery with intimal thickening. In the normal closing ductus of the neonatal controls, the media consisted of layers of smooth muscle cells (SMCs) intermingled with layers of elastin. The intima was thin and poor in elastin. By contrast, the media of PDA in BN rats elastin lamellae were absent and the intima contained many elastic fibers. The abnormal distribution of elastin in the PDA of BN rats suggests that impaired elastin metabolism is related to the persistence of the ductus and implicates a genetically determined factor that may link the PDA with aortic fragility.

Animals↗
PubMed2006

Widened coronary arteries in a feverish child.

A 3-year-old girl with fever of unknown origin after a visit to Surinam was seen at our hospital. Signs and symptoms were indicative of either Kawasaki syndrome or an acute viral or (atypical) bacterial illness. No cardiac abnormalities were noted at echocardiography. She was treated with intravenous immunoglobulin and clarithromycin and made a quick recovery. Serologically, the diagnosis of murine typhus was made; a flea-borne rickettsiosis caused by Rickettsia typhi. A follow-up echocardiography 1 week later showed a dilated left coronary artery, which was normal again 4 weeks later. We suggest that this phenomenon was a manifestation of rickettsial vasculitis.

Anti-Bacterial Agents↗
PubMed2006

Polymorphisms in the mannose-binding lectin gene as determinants of age-defined risk of coronary artery lesions in Kawasaki disease.

OBJECTIVE: To evaluate the relationship between polymorphisms in the gene coding for mannose-binding lectin (MBL) and the occurrence of coronary artery lesions (CALs) among different age groups of patients with Kawasaki disease. METHODS: The frequencies of the genotypes, defined as mutations in codons 52, 54, and 57, and the functional promoter variants of the MBL2 gene were determined in 88 patients with acute Kawasaki disease (median age at onset 1.9 years). The possible influence of the MBL2 genotype on the development and progression of CALs in Kawasaki disease was assessed according to age categories and MBL genotypes in univariate and multivariate analyses. RESULTS: In patients younger than age 1 year, we found an increased risk of developing CALs in the presence of a variant MBL2 genotype (P = 0.008). In contrast, in patients older than age 1 year, we found an increased risk of CALs in those patients with the wild-type genotype (P = 0.005). CONCLUSION: Our findings indicate that MBL has an ambiguous role in Kawasaki disease and contributes differently to the pathophysiologic development of CALs, being protective in infants but potentially harmful in patients of older age. The data also imply that the standard treatment of intravenous immunoglobulins to reduce the development of lesions may not be as effective in the very young as it is in the older patients. For the very young, alternative or adjuvant treatment may be indicated, particularly in infants who are MBL-deficient.

Adolescent↗
PubMed2006

Stenting of the vertical vein in obstructed total anomalous pulmonary venous return as rescue procedure in a neonate.

A newborn girl with progressive respiratory distress and cyanosis was found to have severely obstructed supracardiac total pulmonary venous return (TAPVR). Stenting of the stenosis provided immediate and effective relief of the obstruction until corrective surgery was performed. Catheter intervention should be considered in high-risk infants with obstructed supracardiac TAPVR as part of the strategy for preoperative cardiovascular stabilization.

Constriction, Pathologic↗
PubMed2006

Aortic root dysfunctioning and its effect on left ventricular function in Ross procedure patients assessed with magnetic resonance imaging.

BACKGROUND: This study evaluated the diameters and distensibility of the aortic root as well as the degree of aortic regurgitation (AR) and its effect on left ventricular (LV) function in patients 8.2 +/- 3.1 years after they underwent the Ross procedure, with a comparison of these parameters between patients and matched healthy subjects. METHODS: Eighteen Ross procedure patients (16 male patients, age [mean +/- SD] 19.2 +/- 3.8 years) and 18 matched healthy subjects (16 male patients, age [mean +/- SD] 19.7 +/- 4.2 years) underwent magnetic resonance imaging. Measurements for diameters (at 4 levels) and the distensibility of the aortic root were performed using a steady-state free precession sequence. Aortic flow was assessed with a velocity-encoded phase-contrast sequence. Left ventricular systolic function was assessed with a gradient-echo sequence in the short-axis plane. Comparison of parameters was performed using the Mann-Whitney U test. Correlations between diameters, distensibility, AR fraction, and LV systolic function were expressed with Spearman rank correlation coefficients. Linear regression analysis was used to identify predictors of LV systolic dysfunction. RESULTS: Aortic root diameters were increased in Ross procedure patients as compared with healthy subjects (mean difference 6.3-11.6 mm, P < or = .02 at all 4 levels). Distensibility of the aortic root was lower in patients (1.9 +/- 1.1 vs 7.8 +/- 3.3 mm Hg(-1), P < .01). An AR fraction > 5% was present in 14 of the 18 patients (mean AR fraction 8% +/- 5% vs 1% +/- 1%, P < .01). Left ventricular ejection fraction was lower in patients (50% +/- 6% vs 57% +/- 6%, P < .01). Dilatation, decreased distensibility, and AR fraction were correlated with impaired LV systolic function (P < .05 for all). The AR fraction predicted impaired LV systolic function (P < .01). CONCLUSIONS: Magnetic resonance imaging shows dilatation and decreased distensibility of the aortic root, AR, and consequent impaired LV systolic function in patients after the Ross procedure.

Adolescent↗
PubMed2005

Intraoperative stenting of pulmonary arteries.

OBJECTIVE: The surgical treatment of pulmonary artery stenoses in small children with complex cardiac lesions can be technically difficult. A hybrid-approach combining corrective surgery and intraoperative stent placement may be complementary in these patients. METHODS: Descriptive study in 11 small children (age: one week to 12 years, median of 12 months, weight: 2.5-20 kg) after previous cardiac surgery. Intraoperative stenting of pulmonary arteries was performed involving paediatric cardiologist and cardiac surgeon. Stenting was combined with repair of pulmonary atresia (n=2), right ventricular outflow and pulmonary artery reconstruction (n=3), unifocalisation of pulmonary arteries (n=1), revision of distal anastomosis of RV-PA-conduit after truncus repair (n=1), revision of distal anastomosis of RV-PA-conduit after AVSD/Fallot repair (n=1), aortic arch patch reconstruction after anatomical correction for transposition of the great arteries (n=1), bidirectional cavopulmonary anastomosis after Norwood I operation for hypoplastic left heart syndrome, (n=1) retrieval of a dislodged stent from the left pulmonary artery (n=1). In seven patients stenting was planned electively while in four patients it took place on an emergency base. RESULTS: No complications occurred during stent implantation. One patient died three weeks postoperatively from diffuse bleeding due to a coagulation disorder. Ten patients left hospital after the surgical intervention with concomitant stent implantation. Stent re-dilatation was necessary in 4 patients 2-24 months postoperatively. After a median follow-up of 15 months ranging from 3 weeks to 7.5 years all stents were patent as diagnosed by angiography in 6 patients and by colour-Doppler in all other patients. One year after stent placement one stent was removed and another surgically opened during re-operation for conduit replacement in the smallest patient from this series. There was one late death during operative right ventricular outflow-tract reconstruction after initial stent placement. CONCLUSIONS: With intraoperative stent placement surgically difficult patch augmentation of small and fragile pulmonary vessels during repair of complex cardiac lesions can be avoided. Stents recruit pulmonary vessels and keep them open and amenable to future percutaneous or surgical interventions.

Arterial Occlusive Diseases↗
PubMed2005

Results of the Ross operation in a pediatric population.

OBJECTIVE: To analyse the results of the mid-term clinical and echocardiographic follow-up of the pediatric Ross operation. METHODS: Echo-Doppler follow-up of 53 consecutive pediatric Ross procedures performed between 1994 and 2003. Median age was 9.7 years at time of operation (2 weeks-17.7 years). Six patients were younger than 3 months. Median age at follow-up was 15.6 years. Aortic valve/left ventricular outflow tract (LVOT) anomalies were congenital in 49 (92%). Seventy percent had previous surgery or balloon valvuloplasty. Root replacement was used in all. Thirteen patients (25%) had LVOT enlargement. Mean cross-clamp time was 113 (69-189) minutes. RESULTS: Early mortality occurred in 3 patients after emergency surgery following balloon failure (n=1) and extended Ross following interrupted arch/VSD repair (n=2). Late mortality was due to LV fibroelastosis in 2 patients and complicated pulmonary artery stenting in another. RVOT reoperations were required because of late homograft obstruction in 2 patients and because of pulmonary artery stenosis in another. Five patients (9.4%) were reoperated for pulmonary autograft dilatation (n=3) and for leaflet fibrosis or perforation (n=2). Autografts were repaired in two patients, while a mechanical valve was inserted in 3 cases. At 9 years the actuarial survival and event free survival were 89 and 74%, respectively. At last follow-up 90% of autograft diameters indexed to body surface area was above the 90th percentile of normal aortic root diameters. LVOT and RVOT gradients were low and autograft insufficiency was trivial to mild in 84% and mild to moderate in 16%. Autograft stenosis was not noticed. CONCLUSIONS: The pediatric Ross procedure remains an important tool but autograft dilatation also occurs in the pediatric population. The significance of this finding has yet to be determined.

Adolescent↗
PubMed2005

Management of asymptomatic anthracycline-induced cardiac damage after treatment for childhood cancer: a postal survey among Dutch adult and pediatric cardiologists.

Asymptomatic anthracycline-induced cardiac damage (A-CD) is a serious problem among young childhood cancer survivors. The aim of this survey was to assess the current treatment policy in these patients in the Netherlands. A questionnaire was sent to all 136 departments of adult or pediatric cardiology in the Netherlands. It was returned by 61% of the departments. Sixty-six percent of the respondents started medical treatment (ie, an ACE inhibitor and/or a beta-blocker) in childhood cancer survivors with asymptomatic A-CD. Fifty-eight percent of the respondents indicated that their treatment decision was based on published findings in the literature, but none of them referred to studies evaluating the treatment of asymptomatic A-CD. A majority of adult and pediatric cardiologists started medical treatment in childhood cancer survivors with asymptomatic A-CD without knowledge of the benefits and risks of treatment in this patient group. Before ACE inhibitors and/or beta-blockers can be recommended as routine practice in childhood cancer survivors with asymptomatic A-CD, randomized controlled trials should be performed. Until then, the authors recommend centralizing the treatment of childhood cancer survivors with asymptomatic A-CD in a specialized center to cluster the available knowledge and experience.

Adult↗
PubMed2005

Development of the cardiac conduction system in atrioventricular septal defect in human trisomy 21.

In patients with atrioventricular septal defect (AVSD), the occurrence of nonsurgical AV block has been reported. We have looked for an explanation in the development of the AV conduction system. Human embryos with AVSD and trisomy 21 and normal embryos were examined (age 5-16 wk gestation). Antibodies to human natural killer cell-1 (HNK-1), muscle actin (HHF-35), and collagen VI were used to delineate the conduction system. As in normal hearts, HNK-1 transiently stains the AV conduction system, the sinoatrial node, and parts of the sinus venosus in AVSD. A large distance is present between the superior and inferior node-like part of the right AV ring bundle, comparable to 6-wk-old normal hearts. The definitive inferior AV node remains in dorsal position from 7 wk onward and does not appose to the superior node-like part as seen in normal hearts. Furthermore, in AVSD, a transient third HNK-1-positive "middle bundle" branch that is continuous with the retroaortic root branch and the superior node-like part can be identified, and thus the AV conduction system forms a figure-of-eight loop. At later stages, the AV node remains in dorsal position close to the coronary sinus ostium with a long nonbranching bundle that runs through thin fibrous tissue toward the ventricular septum. The formation of the AV node and the ventricular conduction system in AVSD and Down syndrome differs from normal development, which can be a causative factor in the development of AV conduction disturbances.

Down Syndrome↗
PubMed2004

Predictors of sudden cardiac death after Mustard or Senning repair for transposition of the great arteries.

OBJECTIVES: The goal of this research was to identify predictors for sudden death (SD) in patients with transposition of the great arteries (TGA) who have undergone atrial inflow repair. BACKGROUND: Sudden death is the most common cause of late death after atrial inflow repair of TGA. Little is known about the predictors of SD. METHODS: This was a retrospective, multicenter, case-controlled study. We identified 47 patients after Mustard's or Senning's operation who experienced an SD event (34 SD, 13 near-miss SD). Each patient was matched with two controls with the same operation, but without an SD event. Information on numerous variables before the event was obtained and compared with controls at the same time frame. RESULTS: Presence of symptoms of arrhythmia or heart failure at most recent follow-up and history of documented arrhythmia (atrial flutter [AFL]/atrial fibrillation [AF]) were found to increase the risk of SD. Electrocardiogram (ECG), chest X-ray, and Holter ECG findings were not predictive of SD. Neither medication nor pacing was found to be protective. Most SD events (81%) occurred during exercise. Ventricular tachycardia/ventricular fibrillation were the recorded rhythm during SD in 21 of 47 patients. CONCLUSIONS: Presence of symptoms and documented AFL/AF are the best predictors of SD in TGA patients. Patients with these findings should be further evaluated for risk of SD.

Arrhythmias, Cardiac↗
PubMed2004

Right ventricular function after pulmonary valve replacement in patients with tetralogy of Fallot.

PURPOSE: To assess the time course of right ventricular (RV) function improvement after pulmonary valve replacement (PVR) in patients 25.2 years +/- 7.0 after repair of tetralogy of Fallot. MATERIALS AND METHODS: The medical ethics committee approved this study, and informed consent was obtained. Cardiac magnetic resonance (MR) imaging was performed before, 7 months after, and 19 months after PVR in 25 consecutive patients with tetralogy of Fallot with a 1.5-T MR imager. RV function was assessed with gradient-echo sequences in the short-axis plane. Pulmonary flow was assessed with a velocity-encoded phase-contrast sequence. Paired t test was used to evaluate follow-up data. Independent samples t test was used to assess differences based on the presence of recurrent pulmonary regurgitation (PR). RESULTS: Mean indexed RV end-diastolic volume decreased from 166.9 mL/m(2) +/- 41.3 before PVR to 113.5 mL/m(2)+/- 35.7 (P < .001) at 7-month follow-up and 111.7 mL/m(2)+/- 41.1 (P = .46) at 19-month follow-up. The RV ejection fraction was corrected for PR and improved from 25.0% +/- 7.7 before surgery to 44.1% +/- 11.9 (P < .001) and 45.2% +/- 11.1 (P = .39), at 7- and 19-month follow-up, respectively. Recurrent PR after PVR was found in 11 patients; 14 patients did not have recurrent PR. Total reduction of indexed RV end-diastolic volume at 19 months follow-up was more prominent in patients who did not have recurrent PR than in patients who did have recurrent PR (P < .05). Furthermore, improvement of RV ejection fraction corrected for regurgitation was more marked in patients who did not have recurrent PR than in patients who did have recurrent PR (P < .05). CONCLUSION: In patients with tetralogy of Fallot, RV function improves rapidly after PVR and is sustained at 19-month follow-up in most patients; however, recurrence of PR after PVR appears to reduce recovery of RV systolic function.

Adolescent↗
PubMed2003

Deficiency of the vestibular spine in atrioventricular septal defects in human fetuses with down syndrome.

Data on the morphogenesis of atrioventricular septal defect (AVSD) in Down syndrome are lacking to support molecular studies on Down syndrome heart critical region. Therefore, we studied the development of complete AVSD in human embryos and fetuses with trisomy 21 using 3-dimensional graphic reconstructions and immunohistochemical markers. Eight trisomic hearts with AVSD and 10 normal hearts, ranging from 5 to 16 weeks' gestation, were examined. In AVSD, the muscular septum primum and venous valves develop normally, and the size and histology of the nonfused endocardial cushions also appear normal. However, the mass of extracardiac mesenchyme (vestibular spine), located at the dorsal mesocardium, is reduced and does not protrude ventrally along the right wall of the common pulmonary vein. As a result of this, the muscular septum primum and the right pulmonary ridge are seen as 2 separate septa that attach to the inferior endocardial cushion. Both the muscular septum primum and the superiorly fused venous valves (septum spurium) converge and are capped by a small rim of mesenchyme, which forms the roof of the persisting ostium primum and connects to cushions and the reduced vestibular spine. At 7 weeks, ventricular septation in AVSD is comparable to 5 to 6 weeks of normal cardiac development. At later stages, the septum spurium forms the anterosuperior limbus of the septum secundum and the mesenchymal cap becomes the bridging tendon that connects the bridging leaflets. Therefore, reduced expansion of the vestibular spine derived from the dorsal mesocardium appears to play an important role in the development of AVSD in Down syndrome.

Crown-Rump Length↗
PubMed2003

Transvenous biventricular pacing in a child after congenital heart surgery as an alternative therapy for congestive heart failure.

Transvenous Biventricular Pacing in Children. Cardiac resynchronization therapy improves short-term and long-term hemodynamics in adult patients with congestive heart failure and left bundle branch block. We describe the feasibility of transvenous biventricular pacemaker implantation in a 6-year-old child with heart failure and wide QRS complex after congenital heart surgery. Myocardial tissue Doppler imaging was used to demonstrate intraventricular dyssynchrony and resynchronization after cardiac resynchronization therapy. During 1-year follow-up, symptomatology and hemodynamic parameters improved.

Cardiac Pacing, Artificial↗
PubMed2002

Major coronary anomalies in childhood.

UNLABELLED: Major coronary artery anomalies are extremely rare in childhood. We wanted to assess the historical and diagnostic features and the therapeutic options of three distinct types of coronary artery anomalies: abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA), coronary fistula and coronary stenosis. In a retrospective study, 33 children with these types of coronary artery disease were identified, 15 with ALCAPA, 12 with fistula and six with coronary stenosis. History, physical examination, ECG, X-ray, echocardiography, angiography, therapy and outcome were reviewed. ALCAPA showed distinct typical echocardiographic characteristics. Coronary artery fistula could be identified by a typical murmur and echocardiographic evidence of coronary dilatations. Coronary stenosis should be suspected by the clinical picture in a specific context. CONCLUSION: rare coronary artery anomalies can be accurately diagnosed in childhood. Timely therapy yields good prognosis.

Child, Preschool↗
PubMed2002

Magnetic resonance imaging to assess the hemodynamic effects of pulmonary valve replacement in adults late after repair of tetralogy of fallot.

BACKGROUND: Pulmonary regurgitation (PR) late after total correction for tetralogy of Fallot may lead to progressive right ventricular (RV) dilatation and an increased incidence of severe arrhythmias and sudden death. Timing of pulmonary valve replacement (PVR) is subject to discussion, because the effect of PVR on RV function in adults is unclear. In this study, MRI was used to assess the effect of PVR on RV function and PR. Clinical improvement was established by means of the NYHA classification. METHODS AND RESULTS: Twenty-six adult patients were included. Cardiac MRI was performed at a median of 5.1+/-3.4 months before and 7.4+/- 2.4 months after PVR. Mean preoperative PR was 46+/-10% (range, 25% to 64%). After PVR, 20 of 26 patients (77%) showed no residual PR, 5 patients showed mild residual PR, and 1 patient showed moderate PR. RV end-diastolic volume (RV-EDV) decreased from 305+/-87 to 210+/-62 mL (P<0.001), and RV end-systolic volume (RV-ESV) decreased from 181+/-67 to 121+/-58 mL (P<0.001). No significant change was found in RV-EF (42% versus 42%). However, RVEF corrected for regurgitations and shunting increased from 25.2+/-8.0% to 43.3+/-13.7% (P<0.001). Mean validity class improved from 2.0 to 1.3 (P<0.001). CONCLUSIONS: In adult patients with PR and RV dilatation, late after total correction of tetralogy of Fallot, MRI measurements show remarkable hemodynamic improvement of RV function after PVR and improvement of validity. We therefore advocate a less restrictive management concerning PVR in these patients.

Adolescent↗
PubMed2002

Employment in adults with congenital heart disease.

OBJECTIVE: To evaluate job participation, career-related problems, and actual job problems in adults with complex congenital heart disease (CHD) compared with adults with mild CHD and reference groups. DESIGN: Cross-sectional study. SETTING: Patients were randomly selected from the archives of the Department of Pediatric Cardiology, Leiden University Medical Center, Leiden, the Netherlands. PATIENTS AND MAIN OUTCOME MEASURES: In total, 76 patients with complex CHD and 80 with mild CHD (age range, 17-32 years) completed a self-reported questionnaire on employment and handicaps, with reference data available (response rate, 70%). RESULTS: In the study groups, 45 (59%) of 76 patients with complex CHD had a paid job compared with 61 (76%) of 80 patients with mild CHD. Patients older than 25 years with complex CHD had significantly lower job participation (64%) than the general population (83%). Multiple logistic regression showed that type of CHD and level of education were significantly and independently related to job participation (odds ratio, 4.8; 99% confidence interval, 1.2-19.6; and odds ratio, 4.7; 99% confidence interval, 1.3-17.2, respectively). Of the 76 patients with complex CHD, 42 (55%) experienced disease-related career problems, in contrast to only 1 patient with mild CHD. Both CHD groups had more job-related mobility handicaps than did the reference group. However, in the mild CHD group, handicaps could be attributed to additional noncardiac diseases. CONCLUSIONS: Patients with complex CHD have reduced job participation compared with patients with mild CHD and the general population. Many receive disability benefits or experience career problems or job handicaps. Career counseling focusing on physical abilities and level of education may help prevent or reduce these job-related problems.

Adolescent↗