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We report the case of a long-segment aortic atresia as the cause for therapy resistant arterial hypertension in a young adult. Recanalization was achieved interventionally by wire-crossing and stent implantation with subsequent normalization of blood pressure.
Limited data exist on the vitamin D status in Fontan patients. We determined the prevalence and potential risk factors of vitamin D deficiency in this patient subset.
Methods and results
Data were collected from 27 Fontan patients (55.6% male, mean age 8.1±5.3 years). Protein-losing enteropathy was diagnosed in six patients (22.2%). Vitamin D deficiency was defined as a serum 25-hydroxyvitamin D level of <20 ng/ml. The neutrophil-to-lymphocyte ratio, a marker of systemic inflammation, was calculated. Associations between laboratory measurements and patient characteristics were explored. Mean serum 25-hydroxyvitamin D level was 14.1±10.4 ng/ml. Vitamin D deficiency was found in 19/27 patients (70.3%). Only skin type was associated with vitamin D deficiency (p=0.04). Hyperparathyroidism was present in 5/21 (23.8%) patients, and was more prevalent in patients with protein-losing enteropathy (p<0.001). Parathyroid hormone levels correlated with parameters of systemic inflammation (neutrophil-to-lymphocyte ratio: r=0.484, p=0.026; relative lymphocyte count: r=−0.635, p=0.002). Vitamin D supplementation significantly increased serum 25-hydroxyvitamin D levels (p<0.0001), and was accompanied by a reduction in parathyroid hormone concentrations (p=0.032).
A high prevalence of vitamin D deficiency was found among Fontan patients, independent of age, time after Fontan procedure, ventricular morphology, and presence of protein-losing enteropathy. A potentially important link between parathyroid hormone levels and systemic inflammation is suggested.
We describe transcatheter therapy for early onset occlusion or stenoses of extracardiac conduits in three children who had undergone Fontan completion. Successful stent implantation was associated with complete resolution of symptoms.
A 10-month-old infant with severe tricuspid valve disease due to staphylococcal bacterial endocarditis, underwent surgical replacement of the valve. The new valve was fashioned using an autologous pericardial patch. Over 3 years of follow-up, the new valve has functioned satisfactorily, with moderately elevated right atrial pressure.
A variety of devices are available for transcatheter closure of unwanted shunts. We describe our experience with the use of the Amplatzer vascular plug IV in a consecutive series of infants.
A total of eight consecutive infants – all born preterm at gestational ages ranging from 24 to 35 weeks – undergoing transcatheter closure of unwanted shunts – persistently patent arterial duct in five patients, an aorta to right atrium fistula in one, multiple aortopulmonary collateral vessels in one, and an azygos vein to left atrium connection in one – are described. Their age, from birth, ranged between 3 and 11 months, and weight between 2.6 and 11.3 kilograms. All devices were delivered using percutaneous arterial or venous vascular access via a large lumen (0.038 inch) 4-French delivery catheter.
All lesions could be successfully occluded using one or more devices. Device diameters ranged between 4 and 8 millimetres, and exceeded the minimum diameter of the target vessel by 1 to 2 millimetres. Successful occlusion was confirmed either directly at angiography or on follow-up echocardiography. Of the infants who were mechanically ventilated prior to the procedure, three could be successfully weaned following closure of the shunt. There were no procedure-related complications.
The new vascular plug IV is cheap and efficacious in closing a variety of shunts in young infants, and warrants further extended clinical application.
Juvenile obesity is associated with cardiovascular risk factors. In adults, cardiovascular risk factors and obesity are associated with a decreased number of endothelial progenitor cells. Higher physical fitness correlates with a lower cardiovascular morbidity and increased endothelial progenitor cells.
CD34 positive, KDR/CD34, CD133/CD34, and CD117/CD34 double positive progenitor cells were measured in 24 obese children and adolescents – 15 female; age: 12.5 plus or minus 2.1 years, body mass index standard deviation score: 2.5 plus or minus 0.5, waist: 88.6 plus or minus 15.0 centimetre, body fat: 24.6 plus or minus 2.2% – participating in the CHILT III programme. Percentage body fat was assessed by skinfold thickness. Peak of oxygen uptake and the respiratory quotient were determined by spiroergometry.
No gender differences were found. CD34 positive and CD117 positive/CD34 positive cells correlated with maximum relative watt performance, r is equal to 0.429 and 0.462; p-value less than 0.05. The peak of oxygen uptake correlated with CD34 positive and CD133 positive/CD34 positive cells, r is equal to 0.458 and 0.456; p-value less than 0.05, while no correlations were found between parameters of weight, body composition, and respiratory quotient with progenitor cells.
A higher physical fitness, but not less body fat or body mass index is associated with a higher number of endothelial progenitor cells. These results support the hypothesis that physical fitness and cardiovascular risk in high-risk populations are inversely related. Further research is warranted to clarify the strength of this association and longitudinal effects of a comprehensive obesity programme.
A neonate weighing 2.8 kilograms underwent the staged hybrid procedure for palliation of hypoplastic left heart syndrome. Within 6 hours following placement of a stent to retain patency of the interatrial communication, the infant developed second degree atrioventricular block, which resolved within 24 hours. Four days later, the patient developed complete atrioventricular dissociation. Removal of the stent was followed by recovery of atrioventricular conduction.
To determine the long-term results in patients with critical aortic stenosis who survive initial intervention, and to identify factors which predict prognosis, we studied patients who underwent intervention between 1979 and 1992 for critical aortic stenosis treated within the first three months of life. Patients with a hypoplastic left ventricle or mitral stenosis who were not considered for a biventricular repair were excluded. Follow-up examination included cross-sectional and Doppler echocardiography. All initial and subsequent patient data were reviewed. Of the 64 patients with critical aortic stenosis, 41 (64%) survived more than one month after initial intervention (surgical valvotomy in 39, balloon valvoplasty in two). These survivors constitute the study group. Mild or moderate residual aortic stenosis or regurgitation without further intervention was found in 28 patients at a median duration of 3.1 years (range 0.2–15.0 years). A poor result with re-intervention (n=6) or death (n=7) occurred in 13 patients. The diameter of the aortic valve at presentation was smaller (p<0.02) in patients with a poor result (median 5.5; range 5–15 mm), than in those with a satisfactory result (median 8.0; range 5–10 mm). Significant residual aortic stenosis was present from the time of initial intervention in nine of the 13 patients (69%) with a poor result. No difference was found in the incidence of a duct-dependent systemic circulation, associated cardiac lesions, mechanical ventilation, acidosis or the use of inotropes preoperatively between patients with a satisfactory or a poor late outcome. Of patients with critical aortic stenosis, 64% survived for more than a month after initial intervention. A small aortic valvar diameter at presentation ( 6 mm) and residual stenosis after initial intervention were important determinants of long-term prognosis.
Discrete subaortic stenosis is an acquired lesion that is rare in infancy. We report an infant in whom the lesion developed within four months after cross-sectional echocardiography had revealed an apparently normal outflow tract. Surgical repair was successfully undertaken.
Four patients with the phenotypic characteristics of Noonan's syndrome presented in infancy with a suspected cardiac defect. The cardiac segmental connections were normal. All four were diagnosed to have valvar pulmonary stenosis by cross-sectional echocardiography, the valve being typically thickened and dysplastic as expected for the syndrome. In addition, all had hypertrophic cardiomyopathy and evidence for important abnormalities of the other cardiac valves. Two of the four patients died following cardiac surgery. Of the other two, one child died during interventional catheterization and another died while awaiting cardiac surgery. The cardiac abnormalities, confirmed at postmortem, were dysplastic pulmonary valve with stenosis (four patients), dysplastic aortic valve (three patients), supravalvar aortic stenosis (one patient), small and dysplastic tricuspid and mitral valves (four patients), hypertrophic obstructive cardiomyopathy affecting both ventricles, with asymmetric septal hypertrophy (four patients), atrial septal defect within the oval fossa (one patient). In addition to the usually described abnormalities of the pulmonary valve and ventricular myocardium, the patients described showed a wide spectrum of valvar dysplasia which has received little attention in the past. If these patients do indeed have Noonan's syndrome, and the evidence supports this contention, then these findings support the hypothesis of a more generalized disorder in the development of the ventricles and valves in the syndrome, which has a variable expression. They also stress the need to look carefully for abnormalities of valves other than the pulmonary valve in patients who present with symptoms in infanscy.
A five day old infant underwent repair of coarctation by resection and end-to-end anastomosis of the proximal and distal segments of the arch. Four weeks later, cross-sectional Doppler echocardiography showed a tight recoarctation proximal to the origin of the left subclavian artery. A dilated proximal left subclavian artery was demonstrated, with continuous flow from it into the descending aorta. These findings were confirmed at catheterization. This “vertebral arterial steal” disappeared following transcatheter balloon angioplasty.
Stenoses of the systemic venous pathways are a potentially dangerous complication after the Mustard operation. The acute and intermediate-term efficacy of balloon dilation of these stenoses has not previously been studied in a large group of patients. A retrospective analysis of clinical and angiographic data, with prospective clinical and echocardiographic follow-up, was performed in a consecutive group of 25 patients (17 male, 8 female) who underwent balloon dilation of the superior or inferior caval venous pathways. For 17 dilations of stenoses in the superior pathway, the mean pressure gradient decreased from 9.1±5.7 to 3.4±2.3 mm Hg (p<0.01), while the diameter of the stenosis increased from 5.9±2.9 to 11.2±3.0 mm (p<0.01). For 25 dilations of the inferior pathway, the mean gradient decreased from 4.3±1.8 to 1.4±1.1 mm Hg (p<0.01), while the diameter of the stenosis increased from 7.3±1.5 to 10.6±2.3 mm (p<0.01). Over a median follow-up of 3.5 years, 16 patients have been without symptoms or signs suggestive of recurrence of stenoses. In the remaining nine patients, 11 further procedures were performed at a median of 3.1 years after initial dilation. In five patients who had previously undergone dilation of the superior (3), inferior (1) or both (1) pathways, further dilations of the same pathway(s) and/or stent implantations were performed. Three patients, including one in whom superior caval venous stenosis had recurred, had developed a new stenosis of a previously undilated pathway, while in two patients no further dilations were required. Balloon dilation offers an alternative to reoperation for stenoses of the systemic venous pathways after the Mustard operation, and appears to provide satisfactory intermediate-term palliation. Recurrences of stenoses in previously dilated pathways, and new stenoses in previously unobstructed pathways, are commonly seen at follow-up. Alternative measures, such as placement of stents, need to be considered in the long-term management of these patients.
A neonate presenting with persistent pulmonary hypertension and severe hypoxemia was treated with nitric oxide administered into a head-box. Improvement of clinical, biochemical and echocardiographic parameters were observed during therapy, suggesting an acute decrease of pulmonary arterial pressure. It is feasible to administer nitric oxide to non-ventilated neonates provided all recommended safety precautions are observed
Stenoses in the pulmonary arterial system can have a significant negative impact on the early postoperative course in infants. Early recognition and aggressive management are mandatory.
Patients and methods
We describe our experience with 8 infants, with ages ranging from 3 to 9 months, weighing from 4.5 to 7.7 kilograms, who presented in the up to 18 days following construction of a shunt from the superior caval vein to the pulmonary arteries with clinical symptoms of obstructed pulmonary flow. We include also 2 infants in whom pulmonary arterial stents were implanted in the operating room. Cardiac catheterization showed significant stenoses or occlusion of the left pulmonary arteries in 9 infants, the right pulmonary arteries in 2, or the superior caval vein in 1, the investigation being prompted by the findings of supraphysiological superior caval venous pressures and systemic hypoxaemia. We implanted a variety of stents mounted on balloons ranging in diameter from 6 to 13 millimetres, with 7 placed across a newly created surgical anastomotic site.
All stenoses were crossed successfully, and stents implanted satisfactorily in all patients, albeit that 1 infant suffered an acute tear of the left pulmonary artery, requiring immediate reoperation. This patient died 72 hours later due to a diffuse coagulopathy. All other patients demonstrated sustained clinical improvement following the procedure. At follow-up, 7 of the 9 survivors have progressed to completion of the Fontan circulation. Redilation of the stents was required in the interim, prior to completion of the Fontan circulation, in 4 of them. In 2 patients, the previously implanted stents were incised during the Fontan completion, permitting placement of the extracardiac Goretex conduit from the inferior caval vein to the pulmonary arteries.
Stents can successfully be implanted perioperatively in the pulmonary arterial system during infancy, and redilated, with improvement in clinical outcome in the majority of those with clinically relevant obstruction.
Their perceptual and motor experiences determine the physical and motor development of children, and impact also on their emotional, psychosocial, and cognitive development. Our aim, therefore, was to evaluate motor development in children with congenitally malformed hearts compared to their healthy peers.
We compared 194 children, with a mean age of 10.0 years, and standard deviation of 2.7 years, representing the entire spectrum of congenital cardiac disease, to a control group of 455 healthy children, having a mean age 9.6 years, with standard deviation of 2.17 years. The bodily coordination test for children was used to examine motor development.
Of the children with congenitally malformed hearts, 26.8% showed moderate, and 31.9% had severe disturbances of motor development, compared to 16.5% and 5.5% of the control group, the p-value for these differences being less than 0.001. The mean motor quotient adjusted for age and gender was lower in the children with congenitally malformed hearts than in their healthy peers, at 79.6, with standard deviation of 18.9 as opposed to 96.6, with standard deviation of 15, this difference having a p-value of less than 0.001. Depending on the presence, and/or the degree, of residual sequels, the children with congenitally malformed hearts were divided into two subgroups, with either no or mild residual sequels, or with significant sequels. The mean motor quotient was lower in those with significant residual sequels, at 75, with standard deviation of 19.3, as opposed to 83, with standard deviation of 17.9, the p-value for this difference being less than 0.01. In both subgroups, the mean motor quotient was lower, with a p-value of less than 0.01, than in the control group.
Our findings show that children with congenitally malformed hearts have deficits in their motor development, these being found in the presence of no or mild sequels, as well as with significant residual sequels. Parental overprotection may contribute to these findings.
Aims: StEP TWO is a school- and family-based intervention consisting of extra lessons, healthy nutrition and physical education for overweight and obese children in primary schools, aimed at reducing body mass index by maintenance or reduction of weight, and improving motor abilities. We analysed differences in changes in anthropometric, cardiovascular and obesity parameters between children who underwent intervention, non-participants in intervention, and controls. Methods: Anthropometric data and waist circumference were recorded for 1678 children; body mass index and body mass index–standard deviation score were calculated. Blood pressure was measured after 5 minutes at rest. 121 overweight and obese children enrolled at 3 schools involved in programmes of intervention were invited to take part; 40 of them completed the programme from November 2003 to July 2004. Of these overweight children, 74 were invited, but did not take part. As controls, we enrolled 155 overweight and obese children from 4 other schools. Results: After the programme, the children involved in intervention showed a lower increase in the body mass index (0.3 plus or minus 1.3 versus 0.7 plus or minus 1.2 kilograms per metre squared) and an approximately three times higher diminution of the body mass index–standard deviation score in comparison with their controls (−0.15 plus or minus 0.26 versus 0.05 plus or minus 0.27). Systolic blood pressure was significantly lowered by 9.5 plus or minus 19.6 millimetres of mercury in those involved in intervention, but increased in the control group by 0.5 plus or minus 16.5 millimetres of mercury. Among those invited but not participating, the increase of the body mass index (0.5 plus or minus 1.3 kilograms per metre squared) was less, and the reduction of the body mass index-standard deviation score (−0.09 plus or minus 0.31) and systolic blood pressure (−5.3 plus or minus 15.6 millimetres of mercury) was higher than in the control group. Overweight but not obese children seem to benefit from a screening examination alone. Conclusions: Early preventive measures in schools are necessary and effective for overweight and obese primary school children. The screening itself seems also to have a minor positive effect, especially for overweight children. Sustainability of the observed improvements over a longer period remains to be confirmed.
Obesity in childhood, which is associated with cardiovascular risk factors such as hypertension, is on the increase. Countermeasures are necessary. In this paper, we present the baseline and final data from the StEP TWO programme, a prospective study to prevent overweight and obesity in primary schools. Methods: We recorded and calculated, from 1689 children, anthropometric data, including analyses of bioelectric impedance, waist and hip circumferences, body mass index and its standard deviation, and the ratio of waist to hip. Blood pressure was measured after 5 minutes at rest. From the three schools involved in a programme of intervention, 121 children were invited to take part, and 40 (33.1 per cent) completed the programme. The effect was compared with 155 overweight and obese children identified at the 4 control schools. Results: 830 (49.5 per cent) boys and 848 girls (50.5 per cent) took part. Their mean age was 8.2 plus or minus 1.3 years, their height was 1.31 plus or minus 0.09 metres, they weighed 30.0 plus or minus 8.2 kilograms, and their mean index of body mass was 17.1 plus or minus 2.9 kilograms per metre squared. Of the children, 7.3 per cent were obese, 10.4 per cent were overweight, 75.7 per cent had normal weights, and 6.6 per cent were underweight. Resting hypertension was observed in 2.3 per cent of the children. Increased blood pressure was associated with a higher body weight, body mass index, standard deviation score for body mass index, and waist and hip circumferences (each p < 0.001), but not with the ratio of waist to hip. Hypertension at rest was also found in 11.0 per cent of obese children, 4.4 per cent of those who were overweight, 1.2 per cent of those with normal weight, and 1.0 per cent of underweight children (p < 0.001). After the intervention, the increase of the body mass index tended to be lower in those in whom we had intervened (p = 0.069), and in these the decrease of the standard deviation score for body mass index was significantly higher (p = 0.028). Systolic blood pressure was reduced by about 10 millimetres of mercury in those in whom we had intervened (p = 0.002), while there were no changes in the control group. Diastolic blood pressure was lowered by 3 millimetres of mercury, but this was not significant. Conclusion: Obese children had the highest values for systolic and diastolic blood pressure. Increased levels of blood pressure are associated with other parameters of obesity, such as the circumference of the waist and hip. Early preventive measurements in childhood are necessary, and appropriate intervention appears to be effective.