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PURPOSE: To prospectively assess aortic dimensions, aortic elasticity, aortic valve competence, and left ventricular (LV) systolic function in patients after the arterial switch operation (ASO) by using magnetic resonance (MR) imaging. MATERIALS AND METHODS: Informed consent was obtained from all participants for this local ethics committee-approved study. Fifteen patients (11 male patients, four female patients; mean age, 16 years +/- 4 [standard deviation]; imaging performed 16.1 years after surgery +/- 3.7) and 15 age- and sex-matched control subjects (11 male subjects, four female subjects; mean age, 16 years +/- 4) were evaluated. Velocity-encoded MR imaging was used to assess aortic pulse wave velocity (PWV), and a balanced turbo-field-echo sequence was used to assess aortic root distensibility. Standard velocity-encoded and multisection-multiphase imaging sequences were used to assess aortic valve function, systolic LV function, and LV mass. The two-tailed Mann-Whitney U test and Spearman rank correlation coefficient were used for statistical analysis. RESULTS: Patients treated with the ASO showed aortic root dilatation at three predefined levels (mean difference, 5.7-9.4 mm; P

OBJECTIVE: To investigate whether the association between the maternal methylation status as reflected by low S-adenosylmethionine and high S-adenosylhomocysteine, is detrimental for cardiogenesis and congenital heart disease (CHD) in the offspring. METHODS: As part of a case-control study in the western part of the Netherlands, we evaluated 231 mothers of children with CHD and 315 control mothers of nonmalformed children. The total case group was analyzed and stratified into isolated (n=180) and nonisolated CHDs (n=51). The latter subgroup was further subdivided into Nonsyndromic (n=20), Down Syndrome (n=19), and Other Syndromes (n=12). A multivariable general linear model was used to test for differences between the case groups and controls. All analyses were adjusted for current B vitamin supplement use. RESULTS: Plasma total homocysteine was significantly different between the total case group (median, range 10.3, 4.0-43.8, P=.026) and the nonisolated cases (11.1, 5.5-43.8, P=.006) compared with the controls (10.0, 5.3-42.0). The subgroup of Down Syndrome presented significantly higher total homocysteine and S-adenosylhomocysteine levels and a lower S-adenosylmethionine/S-adenosylhomocysteine ratio than controls. CONCLUSION: Maternal hyperhomocysteinemia, and not hypomethylation, is a risk factor for having a child with CHD. Maternal hypomethylation, however, seems to be associated with offspring having CHD and Down syndrome.

There are very few published reports of the transcatheter closure of perimembranous ventricular septal defects (PMVSDs) using the Amplatzer PMVSD occluder with encouraging initial results. This report presents initial and 1-year results from 54 patients with PMVSDs who underwent transcatheter closure at 5 different institutions with the Amplatzer PMVSD occluder. Sixty-five patients with PMVSDs were enrolled at 5 European centers. Eleven of the 65 patients did not fulfill the patient selection criteria at the initial echocardiographic evaluation or at cardiac catheterization. As a result, a total of 54 patients underwent attempted transcatheter closure using the Amplatzer PMVSD occluder. The median age of the patients was 5.1+/-3.6 years (range 0.3 to 13), and the median weight 18.5+/-10.3 kg (range 5 to 45). Devices were permanently implanted in 49 of 54 patients. Complete occlusion of the communication at 1-year follow-up was observed in 46 of 49 patients (94%). Main early procedural complications included (1) device embolization (2 patients), (2) severe bradycardia with hemodynamic compromise (2 patients), and (3) Mobitz II (2:1) heart block (1 patient). Late procedural complications included complete heart block (1 patient). No other complications were observed during follow-up. In conclusion, the Amplatzer PMVSD occluder is promising device that can be used for transcatheter closure in selected patients with PMVSDs. Further studies and long-term follow-up are required before this technique enters routine clinical practice.

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.

An aortopulmonary window (APW) is a communication between the ascending aorta and the pulmonary trunk in the presence of two separate semilunar valves. In order to increase our understanding about the surgical management of this rare lesion and its long-term results, we describe our experience over a 37-year period. Between 1968 and 2005, 18 patients were diagnosed with APW. Seventeen underwent surgical correction. Age at operation ranged from 22 days to 22 years (median, 0.20 years). Follow-up ranged from 2 weeks to 28.6 years (median, 11.0 years). Surgical closure was achieved using a single patch in 7 patients (41.2%) double patch in 4 (23.5%), primary closure in 3 (17.6%), clip in 2 (11.8%), and ligation in 1 (5.9%). Complex APW was present in 8 patients (44.4%). One patient was treated nonsurgically. There were no early or late deaths after surgery. Both primary closure and patch closure gave excellent long-term results. Sporadic postoperative complications were only associated with complex lesions. One patient who was treated conservatively died (of pulmonary hypertension) 21 years after diagnosis. Repair of APW is ideally performed in the first months of life, before irreversible PHT has developed. Various surgical repair techniques in this series of patients gave excellent short-term and long-term results, without significant hemodynamic sequelae.

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. Copyright

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.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).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).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.

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/m2; left ventricular end diastolic volume, 83 (16) v 69 (10) ml/m2), as was right ventricular wall mass (24 (7) v 17 (2) g/m2). 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.

The lower extremities of 28 unselected children with congenital heart disease were investigated and classified according to the criteria for postthrombotic syndrome five to ten years after their first cardiac catheterization. For the clinical criteria, all patients completed a questionnaire and underwent a standardized physical examination of both legs. For the pathophysiologic criteria, the presence of venous outflow obstruction and reflux was evaluated by color duplex sonography in 24 of the 28 patients. Mild postthrombotic syndrome was present in half the patients. Partial or complete occlusion of the investigated vein was found in four patients (17%). In all patients studied, the venous valves of the deep system were competent. Postthrombotic syndrome frequently occurs in children with congenital heart disease. Prospective studies seem to be justified to investigate the precise incidence and potential risk factors.

Objective The purpose of the present study was to determine the symptomatology, diagnostic procedures, and therapeutic strategies of patients with an isolated unilateral absence of a pulmonary artery (UAPA). Background Isolated UAPA is a rare anomaly. Some case reports exist, but the best diagnostic and therapeutic approaches to these patients remain unclear. Methods A retrospective analysis was made of 108 cases reported between 1978 and 2000. The database of the National Library of Medicine (MEDLINE) was used to identify cases that were published in any language from 1978 onward. Results Of the 108 patients identified, 14 were asymptomatic. The median age was 14 years (range, 0.1 to 58 years). Most patients had symptoms such as frequent pulmonary infections (37%), dyspnea or limited exercise tolerance (40%), or hemoptysis (20%). Pulmonary hypertension was present in 44% of the patients. Surgical procedures were performed in 17% of patients, and the overall mortality rate was 7%. Conclusion Only a few patients with isolated UAPA remain asymptomatic during follow-up. The diagnosis can be made by chest radiograph, echocardiography, CT scan, and MRI. Hilar arteries can be shown by cardiac catheterization and pulmonary venous wedge angiography. This is important since revascularization may improve pulmonary hypertension. The avoidance of high altitudes and pregnancy may further improve outcomes.

Objectives: To evaluate difficulties in daily life, and satisfaction with level of knowledge about their disease, in patients with congenital cardiac disease in order to improve counselling. Methods: A self-administered questionnaire was completed by 80 patients with mild, and 76 with complex, congenital cardiac disease. They were aged from 17 to 32 years. Results: Even patients with only mild malformations experienced difficulties related to their disease, but being found in only 11%, these were significantly less than those uncovered in 87% of those with complex disease (p < 0.001). Those patients with complex malformations frequently felt restricted in choices because of their disease in areas such as sport (59%), employment (51%), and education (34%). Other difficulties reported were: paying a higher premium for life insurance (29%), having to give up on a sport (28%), and being excluded from a job (18%). Depending on the item, between one-fifth and two-thirds of participants reported gaps in knowledge, most frequently for “causes of congenital cardiac disease”, “future consequences”, and “family planning”. For 53% of those with mild anomalies, and 93% of severely affected patients, the cardiologist is the most important source of information. Conclusions: A minority of adults with mild, and a majority of those with complex congenital cardiac disease report difficulties in daily life. A substantial number of these patients feel that they have an inadequate level of knowledge about their disease. Our results suggest the need for a specific programme of counselling.

OBJECTIVE: To examine the impact of previously operated complex congenital heart disease on health related quality of life and subjective health status and to determine the relation between these parameters and physical status. DESIGN: Cross sectional; information on medical follow up was sought retrospectively. SETTING: Patients were randomly selected from the archives of the paediatric cardiology department, Leiden University Medical Centre, Leiden, The Netherlands, and approached irrespective of current cardiac care or hospital of follow up. PATIENTS: Seventy eight patients with previously operated complex congenital heart disease (now aged 18-32 years) were compared with the general population. MAIN OUTCOME MEASURES: Health related quality of life was determined with a specifically developed questionnaire (Netherlands Organisation for Applied Scientific Research Academic Medical Centre (TNO-AZL) adult quality of life (TAAQOL)) and subjective health status was assessed with the 36 item short form health survey (SF-36). Physical status was determined with the objective physical index, Somerville index, and New York Heart Association functional class. RESULTS: Health related quality of life of the patients was significantly worse than that of the general population in the dimensions gross motor functioning and vitality (p < 0.01). Correlations between health related quality of life and physical status were poor. Patients had significantly worse subjective health status than the general population in the dimensions physical functioning, role functioning physical, vitality, and general health perceptions (p < 0.01). Correlations between subjective health status and physical indices were weak. CONCLUSION: Adult survivors with previously operated complex congenital heart disease experienced limitations only in the physical dimensions of health related quality of life and subjective health status. Objectively measured medical variables were only weakly related to health related quality of life. These results indicate that, when evaluating health related quality of life, dedicated questionnaires such as the TAAQOL should be used.

We performed transesophageal echocardiography (TEE) with pediatric biplane probes throughout the procedure of transcatheter closure of the ductus arteriosus in 42 out of 46 patients who were scheduled for this intervention. Patient ages ranged from 15 months to 14 years and body weight from 9-53 kg. Both Rashkind double umbrella devices and coils were used. The described approach resulted in fewer contrast injections and was helpful in monitoring the procedure and in deciding when to release the device. To the best of our knowledge this is the first report describing a large series where continuous monitoring of transcatheter ductus closure was combined with x-ray fluoroscopy and TEE.

The present study examined the social impediments experienced by young adults with minor congenital heart disease (CHD) and compared the health status and health-related quality of life of these CHD-patients with a matched group (age, sex) from the general population. Young adults with minor CHD, not in need of surgical correction, were sent a questionnaire. Results showed that a substantial part of the CHD-patients experienced social impediments, i.e. at school (19%), during free time (15%), in choosing (13%) or performing a job (9%), with medical examinations for job/insurance (19%), and taking out a life insurance policy (8%). Those CHD-patients that experienced social impediments reported a health status and health-related quality of life comparable with the general population. Remarkably, CHD-patients without social impediments reported a better health-related quality of life for 6 of the 12 scales, when compared with the general population. This last result might be explained by coping mechanisms resulting in a higher appreciation of health status which is expressed in a higher health related quality of life.

Background —Abnormal atrial automaticity in young patients with structurally normal hearts is often located around the pulmonary veins and in sinus venosus–related parts of the right atrium. We hypothesize that these ectopic pacemaker sites correspond to areas of embryonic myocardium with an early phenotypic differentiation, as indicated by differences in antigen expression during normal cardiac development. Methods and Results —In human embryos ranging in age from 42 to 54 days of gestation, the development of the cardiac conduction system was studied with the use of HNK-1 immunohistochemistry. HNK-1 stains the developing atrioventricular conduction system, ie, the bundle branches, His bundle, right atrioventricular ring, and retroaortic ring. In addition, the myocardium around the common pulmonary vein showed transient HNK-1 antigen expression. In the right atrium, 3 HNK-1–positive connections were demonstrated between the sinoatrial node and the right atrioventricular ring. An anterior tract through the septum spurium connects the sinoatrial node with the anterior right atrioventricular ring, and 2 posterior tracts connect the sinoatrial node with the posterior right atrioventricular ring through the right venous valve (future crista terminalis) and sinus septum, encircling the coronary sinus. The medioposterior part of the right atrioventricular ring connected to the His bundle and the medioanterior part form 2 node-like structures. Conclusions —In patients with abnormal atrial automaticity, the distribution of left and right atrial pacemaker foci correspond to areas of the embryonic myocardium that temporarily express the HNK-1 antigen.

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.

In clinical treatment of children with congenital heart disease (CHD) assessment of right ventricular (RV) function is important. Available imaging techniques have been of limited value because of technical factors and the complex geometry of the right ventricle. To validate magnetic resonance (MR) imaging measurements of RV function in children, gradient echo MR imaging of both ventricles and MR flow mapping of great vessel and tricuspid flow was performed in 20 children with CHD affecting the right ventricle and in 22 healthy children ranging in age from 5 to 16 years. Close correlation between RV versus LV stroke volumes ( r = 0.96) and RV stroke volume versus great artery ( r = 0.97) or tricuspid flow ( r = 0.97) was observed with small interobserver and intraobserver variability. Results of healthy children were end-diastolic volume: 70 ± 9 ml/m 2 , end-systolic volume: 21 ± 5 ml/m 2 , and ejection fraction: 70% ± 4%. In the patient groups clinically important differences were noted. We conclude that MR imaging provides accurate noninvasive measurements of RV function in healthy children and patients with (operated) CHD.

Few data exists on the differences in long-term outcome between Mustard and Senning operations. We reviewed available data of all hospital survivors of these operations and assessed risk factors for late death and sinus node dysfunction. Of those patients undergoing the Mustard operation, 60 were hospital survivors (46 simple transposition, 14 complex); of those patients undergoing the Senning operation, 62 were hospital survivors (43 simple, 19 complex). Median duration of follow-up was 16 years (maximum 25 years) for Mustard operation, 11 years (maximum 20 years) for Senning operation. No reoperations were done, except for pacemaker implantation. No differences were found between the two groups with regard to baffle-associated problems, right ventricular failure, sudden death (6% in both groups), and functional status at final follow-up (New York Heart Association class I or II, except for four patients). For patients undergoing the Mustard operation, survival at 16-year follow-up was 91% with simple transposition and 60% with complex transposition ( p = 0.027); for both groups of patients undergoing the Senning operation, survival at 16-year follow-up was 78%. Survival in the absence of rhythm disturbance at 16-year follow-up was 18% for Mustard operation and 53% for Senning operation ( p

A 5-month-old infant with aorto-right ventricular tunnel, pulmonary stenosis and a sole supply of the left coronary artery from the tunnel underwent closure of the ventricular end of the tunnel, subsequent to balloon pulmonary valvuloplasty. Care was taken not to obstruct the aortic end of the tunnel in order to maintain the left coronary artery perfusion.

BACKGROUND Pulmonary regurgitation frequently occurs after surgical correction of tetralogy of Fallot. To date, reliable quantitation of pulmonary regurgitation has not been possible, and therefore the clinical significance of pulmonary regurgitation is controversial. Nuclear magnetic resonance (NMR) velocity mapping allows accurate measurement of volumetric flow. The feasibility and accuracy of NMR velocity mapping to quantify pulmonary regurgitation volumes are studied in patients after Fallot repair. METHODS AND RESULTS In 18 patients (mean age, 16.5 +/- 6.5 years), late (12.6 +/- 5.2 years) after Fallot surgery, forward and regurgitant volume flow was measured in the main pulmonary artery with NMR velocity mapping. To validate the measurements of pulmonary forward flow, right ventricular stroke volume was used as an internal reference standard. Pulmonary regurgitation volumes were compared with the differences between the corresponding right and left ventricular stroke volumes. Ventricular volumes were measured with a multisection gradient echo NMR method. In addition, the relation between pulmonary regurgitation and right ventricular volumes was studied. Measurements of pulmonary regurgitation volume with NMR velocity mapping closely corresponded with the tomographically determined volumes (r = .93). Forward pulmonary volume flow was nearly identical to right ventricular stroke volume (r = .98). Pulmonary regurgitation volume was significantly correlated with end-diastolic volume (r = .82, P < .0005), end-systolic volume (r = .63, P < .01), and stroke volume (r = .89, P < .0005) of the right ventricular but not with right ventricular ejection fraction (r = .41, P = NS). CONCLUSIONS NMR velocity mapping is an accurate method for the noninvasive, volumetric quantification of pulmonary regurgitation after surgical correction of tetralogy of Fallot.

From 1958 to 1992, 105 patients were operated for discrete (or membranous) subaortic stenosis (DSAS). Our surgical techniques changed during this period. Before 1980 DSAS was removed by a sharp excision leaving the part on the mitral valve untouched (n = 58). After 1980 blunt dissection was used to enucleate DSAS completely (n = 47). The operation for DSAS was combined with aortic valve replacement (AVR) in 7 instances. Follow-up with recent echo-Doppler studies was complete in 100 patients. Recurring or persisting DSAS led to reoperation in 17 patients. Sixteen of them had had sharp, incomplete resections. One patient from the group of complete blunt enucleations was reoperated on twice. The interval between the first operation and the first reoperation averaged 9.7 years (range: 1-33 years). Nine reoperated patients had AVR. The risk factors for AVR were older age at first operation or late reoperation. In our early experience five patients died after operation. Another three patients died following one or more reoperations. Blunt dissection never led to complete AV block. It follows the anatomical cleavage planes and always allowed for complete and safe removal of DSAS. Although the follow-up is shorter, blunt enucleation thus far has had a lower incidence of reoperation.

ObjectivesTo investigate the effect of bosentan in patients with a failing Fontan circulation.DesignA multicentric open label, non-controlled study.Setting5 tertiary care centres for congenital cardiology.PatientsWe included 10 patients with a failing Fontan circulation. Their median age at inclusion was 12.12 years, with a range from 4.41 to 33,41 years. The median interval between the Fontan operation and inclusion was 7.84 years, with a range from 1.96 to 12,18 years. Participants received half the usual dose of bosentan for 4 weeks, and then the full dose for a further 12 weeks.Main measures of outcomesWe assessed saturations of oxygen at rest and during exercise, using a 6 minutes walk test, at baseline, and during and after 16 weeks of treatment. At each visit, we assessed blood chemistry and hepatic function, and asked the patients to complete a questionnaire concerning quality of life. All medical events and possible side effects were recorded.ResultsOf the cohort, 1 patient withdrew. The changes in saturations of oxygen, exercise performance, and scores for the questionnaire did not reach statistical significance for the whole group. We noted, nonetheless, that saturations of oxygen and/or exercise capacity improved in 5 of the patients. This was further confirmed when those patients deteriorated again when the drug was discontinued.ConclusionsOur study failed to show significant improvement after 3 months of treatment with bosentan in a small group of patients with failing Fontan circulations. Some individuals, nonetheless, did improve. When planning larger trials, it would be better to identify those patients who might potentially benefit from the treatment prior to commencing the trial.

PURPOSE To validate magnetic resonance imaging (MRI) assessment of aortic pulse wave velocity (PWV(MRI)) with PWV determined from invasive intra-aortic pressure measurements (PWV(INV)) and to test the reproducibility of the measurement by MRI. MATERIALS AND METHODS PWV(MRI) was compared with PWV(INV) in 18 nonconsecutive patients scheduled for catheterization for suspected coronary artery disease. Reproducibility of PWV(MRI) was tested in 10 healthy volunteers who underwent repeated measurement of PWV(MRI) at a single occasion. Velocity-encoded MRI was performed on all participants to assess PWV(MRI) in the total aorta (Ao(total)), the proximal aorta (Ao(prox)), and the distal aorta (Ao(dist)). RESULTS The results are expressed as mean +/- SD, Pearson correlation coefficient (PCC), and intraclass correlation (ICC). Good agreement between PWV(MRI) and PWV(INV) was found for Ao(total) (6.5 +/- 1.1 m/s vs. 6.1 +/- 0.8 m/s; PCC = 0.53), Ao(prox) (6.5 +/- 1.3 m/s vs. 6.2 +/- 1.1 m/s; PCC = 0.69), and for Ao(dist) (6.9 +/- 1.1 m/s vs. 6.1 +/- 1.0 m/s; PCC = 0.71). Reproducibility of PWV(MRI) was high for Ao(total) (4.3 +/- 0.5 m/s vs. 4.6 +/- 0.7 m/s; ICC = 0.90, P < 0.01), Ao(prox) (4.3 +/- 0.9 m/s vs. 4.7 +/- 1.0 m/s; ICC = 0.87, P < 0.01), and Ao(dist) (4.3 +/- 0.6 m/s vs. 4.4 +/- 0.8 m/s; ICC = 0.92, P < 0.01). CONCLUSION MRI assessment of aortic pulse wave velocity shows good agreement with invasive pressure measurements and can be determined with high reproducibility.

Parents of children with congenitally malformed hearts can suffer from stress as a result of the medical condition of their child. In this cross-sectional study, we aimed to describe levels of parental stress, and perceived vulnerability, in parents of children who underwent major cardiac surgery, by using both generic and disease-related measures for assessment. We included parents of children who underwent open-heart surgery over the period 2002 through 2007 in the Center for congenital Anomalies Heart Amsterdam/Leiden, abbreviated to provide the acronym CAHAL. In total, we assessed 114 mothers and 82 fathers of 131 children, using the Pediatric Inventory for Parents, short form, General Health Questionnaire, Parental Stress Index-Short Form, State-Trait Anxiety Index and the Child Vulnerability Scale. Compared to the reference groups of the instruments used, parents of children with congenitally malformed hearts did not report higher generic nor disease-related stress scores, and parenting levels of stress were also comparable to reference groups. State anxiety levels, however, were higher in mothers of children with congenitally malformed hearts. Both fathers and mothers reported significantly higher rates of perceived vulnerability than did parents of healthy children. Risk factors for increased anxiety and perceived vulnerability were found in the number of surgical procedures, the time past since the last procedure, and ethnicity. Severity of the lesion did not influence parental levels of stress, but parents of children with hypoplastic left heart syndrome did report higher levels of stress than other parents. Psychosocial screening of parents of children with congenitally malformed hearts is important in order to provide appropriate counselling to those parents most in need.

Background: Aortic wall pathology and concomitant aortic dilatation have been described in tetralogy of Fallot (TOF) patients, which may negatively affect aortic valve and left ventricular systolic function. Objective: To assess aortic dimensions, aortic elasticity, aortic valve competence and biventricular function in repaired TOF patients after pulmonary valve replacement (PVR) using magnetic resonance imaging (MRI). Methods: MRI was performed in 16 patients with TOF after PVR (10 male; mean age 31 years (SD 15)) and 16 age and gender-matched healthy subjects. Results: TOF patients showed aortic root dilatation (mean difference 7.8-8.8 mm, p

With the exception of studies on folic acid, little evidence is available concerning other nutrients in the pathogenesis of congenital heart defects (CHDs). Fatty acids play a central role in embryonic development, and the B-vitamins riboflavin and nicotinamide are co-enzymes in lipid metabolism. Aim of the study To investigate associations between the maternal dietary intake of fats, riboflavin and nicotinamide, and CHD risk in the offspring. Methods A case-control family study was conducted in 276 mothers of a child with a CHD comprising of 190 outflow tract defects (OTD) and 86 non-outflow tract defects (non-OTD) and 324 control mothers of a non-malformed child. Mothers filled out general and food frequency questionnaires at 16 months after the index-pregnancy, as a proxy of the habitual food intake in the preconception period. Nutrient intakes (medians) were compared between cases and controls by Mann-Whitney U test. Odds ratios (OR) for the association between CHDs and nutrient intakes were estimated in a logistic regression model. Results Case mothers, in particular mothers of a child with OTD, had higher dietary intakes of saturated fat, 30.9 vs. 29.8 g/d; P < 0.05. Dietary intakes of riboflavin and nicotinamide were lower in mothers of a child with an OTD than in controls (1.32 vs. 1.41 mg/d; P < 0.05 and 14.6 vs. 15.1 mg/d; P < 0.05, respectively). Energy, unsaturated fat, cholesterol and folate intakes were comparable between the groups. Low dietary intakes of both riboflavin (< 1.20 mg/d) and nicotinamide (< 13.5 mg/d) increased more than two-fold the risk of a child with an OTD, especially in mothers who did not use vitamin supplements in the periconceptional period (OR 2.4, 95%CI 1.4-4.0). Increasing intakes of nicotinamide (OR 0.8, 95%CI 0.7-1.001, per unit standard deviation increase) decreased CHD risk independent of dietary folate intake. Conclusions A maternal diet high in saturated fats and low in riboflavin and nicotinamide seems to contribute to CHD risk, in particular OTDs.

PURPOSE: To prospectively assess right ventricular (RV) function after the Ross procedure by using magnetic resonance (MR) imaging. MATERIALS AND METHODS: The local ethics committee approved the study and informed consent was obtained from all participants prior to enrollment in the study. Seventeen patients (15 male, two female; mean age +/- standard deviation, 19 years +/- 3.9; imaging performed 8.3 years after surgery +/- 3.2) and 17 matched controls (15 male, two female; mean age +/- standard deviation, 20 years +/- 3.9) were studied by using MR imaging. Standard velocity-encoded and multisection multiphase imaging sequences were used to assess homograft valve function, systolic and diastolic RV function, and RV mass. The two-tailed Mann-Whitney U test and the Spearman rank correlation coefficient were used for statistical analysis. RESULTS: Minor degrees of homograft stenosis (peak flow velocity between 1.5 and 3.0 m/sec across the homograft valve) were found in 12 of 17 patients but not in controls (P

We report on two apparently unrelated girls with Johanson-Blizzard syndrome (JBS), in both children caused by a homozygous IVS26 + 5G > A mutation in the UBR1 gene. In both cases the parents are consanguineous and more sibs are affected. The somewhat mild phenotype (with no or slight mental retardation) in these two JBS families might be explained by residual UBR1 activity. One case has a dilated cardiomyopathy, a symptom only rarely reported in JBS, but of important clinical significance. (C) 2008 Wiley-Liss, Inc

Objective. The need for conduit replacement in the growing child remains a major problem after right ventricular outflow tract reconstruction. We compared two diverse surgical centers with considerable practice variation in Europe and the United States to identify modifiable risk factors that can increase conduit longevity. Design. Retrospective analysis of 194 patients (56 Europe, 138 United States) who underwent primary right ventricular to pulmonary artery conduit placement between January 1987 and March 2003. Patients. Diagnoses included tetralogy of Fallot with pulmonary atresia, truncus arteriosus, transposition of the great arteries with ventricular septal defect and pulmonary stenosis, and double-outlet right ventricle. Results. Median age was 7.3 months (range 2 days–29.9 years). Types of conduits included aortic homografts (n = 111), pulmonary homografts (n = 48), Contegra conduits (Medtronic, Inc, Minneapolis, MN) (n = 23), and synthetic conduits (n = 12). Freedom from conduit failure at 5 years was 50% (58% Europe, 48% United States, P = NS). On multivariate analysis, smaller conduit diameter (hazard ratio [HR] 1.15, P

Summary Kawasaki disease (KD) is an acute vasculitis occurring in young children. Its aetiology is unknown, but an infectious agent is assumed. Increased levels of proinflammatory cytokines and chemokines have been reported in KD. Genetic variation in these genes and the receptors for these genes could influence the regulation of cytokines and chemokines. In a case–control study of 170 Dutch Caucasian KD patients and 300 healthy Dutch Caucasian controls, common genetic variants in chemokine receptor genes CCR3, CCR2, CCR5, CX3CR1, CXCR1 and CXCR2 were analysed. Of the eight studied single nucleotide polymorphisms (SNPs) in the CCR3–CCR2–CCR5 gene cluster, four showed a significant association with susceptibility to KD. Moreover the CCR5-Δ32 was observed with an allele frequency of 10·7% in the control population compared to 6·5% in the KD patients (P = 0·04). Two haplotypes of the CCR3–CCR2–CCR5 gene-cluster appear to be at risk haplotypes for KD and one a protective haplotype. No association was observed with the studied SNPs in CX3CR1, CXCR1 and CXCR2. In conclusion, in a Dutch cohort of KD patients an association of KD occurrence with common genetic variants in the chemokine receptor gene-cluster CCR3–CCR2–CCR5 was observed.

OBJECTIVE: To assess pulmonary flow dynamics and right ventricular (RV) function in patients without significant anatomical narrowing of the pulmonary arteries late after the arterial switch operation (ASO) by using magnetic resonance imaging (MRI). METHODS: 17 patients (mean (SD), 16.5 (3.6) years after ASO) and 17 matched healthy subjects were included. MRI was used to assess flow across the pulmonary trunk, RV systolic and diastolic function, and RV mass. RESULTS: Increased peak flow velocity (>1.5 m/s) was found across the pulmonary trunk in 14 of 17 patients. Increased RV mass was found in ASO patients: 14.9 (3.4) vs 10.0 (2.6) g/m2 in normal subjects (p

This study sought to assess elasticity and dimensions of the aorta and their impact on aortic valve competence and left ventricular (LV) function in patients with a nonstenotic bicuspid aortic valve (BAV). Intrinsic pathology of the aortic wall is a possible explanation for reduced aortic elasticity and aortic dilatation in patients with BAVs, even in the absence of a stenotic aortic valve. The relationship between aortic wall elasticity, aortic dimensions, aortic valve competence, and LV function in patients with BAVs has not previously been studied with magnetic resonance imaging. Magnetic resonance imaging was performed in 20 patients with nonstenotic BAVs (mean +/- SD, age 27 +/- 11 years) and 20 matched control patients. The BAV patients showed reduced aortic elasticity as indicated by increased pulse wave velocity in the aortic arch and descending aorta (5.6 +/- 1.3 m/s vs. 4.5 +/- 1.1 m/s, p = 0.01; and 5.2 +/- 1.8 m/s vs. 4.3 +/- 0.9 m/s, p = 0.03, respectively), and reduced aortic root distensibility (3.1 +/- 1.2 x 10(-3) mm Hg(-1) vs. 5.6 +/- 3.2 x 10(-3) mm Hg(-1), p < 0.01). In addition, BAV patients showed aortic root dilatation as compared with control patients (mean difference 3.6 to 4.2 mm, p < or = 0.04 at all 4 predefined levels). Minor degrees of aortic regurgitation (AR) were present in 11 patients (AR fraction 6 +/- 8% vs. 1 +/- 1%, p < 0.01). The LV ejection fraction was normal (55 +/- 8% vs. 56 +/- 6%, p = 0.61), whereas LV mass was significantly increased in patients (54 +/- 12 g/m2 vs. 46 +/- 12 g/m2, p = 0.04). Dilatation at the level of the aortic annulus (r = 0.45, p = 0.044) and reduced aortic root distensibility (r = 0.37, p = 0.041) correlated with AR fraction. Increased pulse wave velocity in the aortic arch correlated with increased LV mass (r = 0.42, p = 0.041). Reduced aortic elasticity and aortic root dilatation were frequently present in patients with nonstenotic BAVs. In addition, reduced aortic wall elasticity was associated with severity of AR and LV hypertrophy

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 5% was present in 14 of the 18 patients (mean AR fraction 8% +/- 5% vs 1% +/- 1 %, P

Objective To investigate whether common genetic variants in the vascular endothelial growth factor (VEGF) gene are associated with Kawasaki disease (KD) and the subsequent development of coronary artery lesions. Methods Common genetic variants in the VEGF gene were analyzed in an association study in a Dutch cohort of 170 KD patients and 300 healthy Dutch Caucasian controls. Genotyping was done with 5′-nuclease TaqMan assays and 3′-hybridization–triggered fluorescence minor groove binder Eclipse assays. Results An association with susceptibility to KD was observed with 2 of the 6 single-nucleotide polymorphisms analyzed in VEGF: –2594 A>C (rs699947) and the 236 bp 3′ of STP C>T (rs3025039). Also for an 18-bp deletion in the promoter of VEGF a significant difference in the genotype and allele frequencies was observed between the KD patients and the controls. The haplotype CGCC (based on rs699947, rs2010963, rs25648, and rs3025039) was significantly associated with the development of KD (hap score 3.8; P = 0.0002). VEGF plasma levels were significantly higher in patients with the early phase of KD than in the healthy controls, and there was a trend toward higher VEGF plasma levels in KD patients with the –2594 CC and 236 bp 3′ of STP CC genotypes. Conclusion Our results suggest that polymorphisms of the VEGF gene may play a role in the pathogenesis of KD.

Aortic valve insufficiency has been described to occur after the arterial switch operation. Valve insufficiency is mild in most cases. In some patients valve repair or root reconstruction may solve the problem, but in others the valve will have to be replaced. Inasmuch as the number of patients surviving the arterial switch operation is growing, valve insufficiency may be seen more often. In infants the ideal way to replace the aortic valve is by using a pulmonary autograft. Our experience with aortic valve replacement using a pulmonary autograft (former aortic valve) in one infant with a history of arterial switch operation is described. Clinical summary. A little girl, 3 years 4 months old, was referred to us from another institution. At the age of 2 days an arterial switch operation was performed for simple transposition of the great arteries. The coronary anatomy was normal (1LCx-2R, Leiden classification). 1 The aorta was anterior to the pulmonary artery. Both semilunar valves were tricuspid and normal. The coronary arteries were excised together with U-shaped aortic wall buttons and implanted in the pulmonary artery base by means of the trapdoor technique. The neopulmonary root was reconstructed with a pantaloon-shaped patch of autologous pericardium. A Lecompte maneuver was part of the procedure. The postoperative course was complicated by ischemia, frequent ventricular extrasystoles, and periods of ventricular fibrillation. Left hemidiaphragmatic paresis necessitated later diaphragmatic plication. After an extended period of intensive care, she gradually recovered and finally was discharged from the hospital. Progressive dilatation of the aortic root with valvular insufficiency was observed during follow-up. Insufficiency was considered to be significant (2/4) when she was 1 year old. The left ventricle was dilated. Her condition deteriorated gradually, leading to fatigue on slight exertion.

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.

Objective: To discuss the need for continuing cardiac surveillance in adults with hemodynamically insignificant congenital cardiac disease. Methods: In 82 patients with mild congenital cardiac malformations, aged from 17 to 32 years, we investigated the subjective health status, the health-related quality of life, any difficulties encountered in daily life, the regularity of follow-up, current diagnosis, and antibiotic prophylaxis. Results: The subjective health status, and the health-related quality of life, did not differ from those of the general population. Nevertheless, patients experienced unnecessary difficulties with choice of sport, obtaining insurance cover, and education. After clinical re-evaluation, diagnosis and antibiotic regimes had to be changed in 9 patients (11%). Conclusions: Patients with a mild congenital cardiac malformation consider themselves to be in good health. So as to fine tune the diagnosis, and update the information supplied to the patients, we suggest that at least a cardiological assessment be carried out at the age of 16 to 18 years. In this way, patients might be protected from unnecessary difficulties, such as restrictions for sport or the charging of unjustifiably high rates for insurance.

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 (nZ2), right ventricular outflow and pulmonary artery reconstruction (nZ3), unifocalisation of pulmonary arteries (nZ1), revision of distal anastomosis of RV-PA-conduit after truncus repair (nZ1), revision of distal anastomosis of RV-PAconduit after AVSD/Fallot repair (nZ1), aortic arch patch reconstruction after anatomical correction for transposition of the great arteries (nZ1), bidirectional cavopulmonary anastomosis after Norwood I operation for hypoplastic left heart syndrome, (nZ1) retrieval of a dislodged stent from the left pulmonary artery (nZ1). 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 redilatation 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. q 2005 Elsevier B.V. All rights reserved.

Objective: To analyse the results of the mid-term clinical and echocardiographic follow-up of the pediatric Ross operation. Methods: EchoDoppler 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 (nZ1) and extended Ross following interrupted arch/VSD repair (nZ2). 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 (nZ3) and for leaflet fibrosis or perforation (nZ2). 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 followup 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. q 2005 Elsevier B.V. All rights reserved.

This study represents the development and validation of a cardiac-specific module of the generic health-related quality of life (HRQoL) instrument, the TAAQOL (TNO/AZL Adult Quality Of Life), for young adults with congenital heart disease (CHD). Items were selected based on literature, an explorative previous study in CHD patients, interviews with patients, and the advice of experts. The newly developed Congenital Heart Disease-TNO/AZL Adult Quality of Life (CHD-TAAQOL) was tested in 156 patients with mild or complex CHD and consisted of three hypothesised subject scales: 'Symptoms' (9 items), 'Impact Cardiac Surveillance' (7 items), and 'Worries' (10 items). Cronbach's alpha for the three scales were 0.77, 0.78, and 0.82, respectively. Scale structure was confirmed by Principal Component Analysis, corrected item-scale and interscale correlations. Overall, 55% of reported health status problems were associated with negative emotions, which is an argument for assessing HRQoL as a concept distinct from health status. Convergent validity with validated generic instruments (TAAQOL and Short Form-36, SF-36) showed satisfactory coefficients. Discriminant validity was proven by significantly higher scores for mild CHD patients compared with those with complex CHD. In conclusion, the CHD-TAAQOL module together with the generic TAAQOL can be used to assess group differences for cardiac-specific HRQoL in young adults with CHD. Testing psychometric properties of the CHD-TAAQOL shows satisfactory results. However, to detect changes in HRQoL over time, further research is needed.

This report describes the mid-term beneficial hemodynamic effect of biventricular pacing in an infant with congestive heart failure after congenital heart surgery, due to resynchronization of the left and right ventricle, optimization of the AV delay, and (partial) correction of the LV dyssynchrony.

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.

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.

The purpose of the present study was to determine the symptomatology, diagnostic procedures, and therapeutic strategies of patients with an isolated unilateral absence of a pulmonary artery (UAPA).Isolated UAPA is a rare anomaly. Some case reports exist, but the best diagnostic and therapeutic approaches to these patients remain unclear.A retrospective analysis was made of 108 cases reported between 1978 and 2000. The database of the National Library of Medicine (MEDLINE) was used to identify cases that were published in any language from 1978 onward.Of the 108 patients identified, 14 were asymptomatic. The median age was 14 years (range, 0.1 to 58 years). Most patients had symptoms such as frequent pulmonary infections (37%), dyspnea or limited exercise tolerance (40%), or hemoptysis (20%). Pulmonary hypertension was present in 44% of the patients. Surgical procedures were performed in 17% of patients, and the overall mortality rate was 7%.Only a few patients with isolated UAPA remain asymptomatic during follow-up. The diagnosis can be made by chest radiograph, echocardiography, CT scan, and MRI. Hilar arteries can be shown by cardiac catheterization and pulmonary venous wedge angiography. This is important since revascularization may improve pulmonary hypertension. The avoidance of high altitudes and pregnancy may further improve outcomes.

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 P P P 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.

Objectives: To analyze the results of pulmonary valve insertion late after initial repair of Fallot’s tetralogy. Pulmonary insufficiency (PI) after correction of Fallot’s tetralogy is usually well tolerated in the short term, but is associated with symptomatic right ventricular dilatation and an increased risk of ventricular arrhythmias over longer periods of time. Methods: From 1993 to July 2000, 51 patients were reoperated for PI at a mean age of 25.7 ^ 11.9 years. The mean age at initial repair was 6.4 ^ 7.2 years. Patients with a conduit inserted at initial operation, with absent pulmonary valve syndrome or with a more than moderate ventricular septal defect at reoperation were excluded from the study. A cryopreserved pulmonary (96%) or aortic (4%) homograft was implanted in the orthotopic position with the use of cardiopulmonary bypass 19.3 ^ 9.1 years (2.7‐40.3 years) after initial correction. Preoperative symptoms (New York Heart Association, NYHA class), degree of PI (echo-Doppler, MRI), right ventricular dimensions (MRI) and QRS duration were compared to findings at last follow-up. Results: Follow-up is complete and had a mean duration of 1.7 ^ 1.4 years. Hospital mortality was 2%. No serious morbidity occurred. Severe PI was present preoperatively in all patients. At last follow-up echo-Doppler studies showed PI to be absent or trivial in 96% and mild in 4% of patients. In 13 patients MRI studies were performed both pre- and postoperatively: in this group PI was reduced from a mean of 48 to 4%. After 6 months NYHA capacity class had improved significantly from 2.3 ^ 0.6 to 1.4 ^ 0.5. After 1 year end-diastolic and end-systolic right ventricular volumes were reduced significantly. Right ventricular ejection fraction and QRS duration remained unchanged. Conclusions: PI late after correction of Fallot’s tetralogy may lead to serious symptomatic right ventricle dilatation. After pulmonary homograft insertion right ventricular dimensions decrease rapidly and functional improvement is observed in almost all patients. q 2001 Elsevier Science B.V. All rights reserved.