Sabine Schickendantz

Motor development in children with congenital cardiac diseases compared to their healthy peers

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.

Prolonged survival in alveolar capillary dysplasia syndrome.

Survival for up to 101 days is reported in an infant with congenital alveolar capillary dysplasia (ACD) with misalignment of pulmonary veins using inhaled nitric oxide thereby offering the prospect of survival until lung transplantation can be performed. The patient was a 3040 g Caucasian girl delivered at 39 weeks gestation. She developed cyanosis, tachypnoea, and hypoxaemia at 12 h of age. Echocardiography demonstrated atrial and ductal right-to-left shunt and suprasystemic right ventricular pressure. Following evaluation of the effect of different dosages of inhaled nitric oxide (iNO) during cardiac catheterisation, the therapeutic dose was chosen to be between 2–10 ppm, with a target O2 saturation of 95%. Right ventricular pressure decreased from 90–110 mmHg before to 60–65 mmHg after starting NO inhalation. Mean systolic arterial blood pressure was 55–75 mmHg. Breathing rate ranged from 30 to 50/min. A sibling had died 5 years earlier at the age of 17 days from a similar clinical presentation and ACD had been diagnosed at autopsy. At the age of 23 days, an open lung biopsy was performed. Pulmonary lobules were abnormally developed and showed thickened immature septa. Alveolar capillaries were frequently located in a central position deep inside the septa and were rarely in contact with the alveolar epithelium. Pulmonary veins could be found within the bronchovascular bundles adjacent to the pulmonary arteries instead of their normal intra-acinar course away from the arterial branches (‘‘misalignment’’). Pulmonary arteries showed thickened muscular walls reflecting persistent pulmonary hypertension. In addition, prematurely muscularised arterioles were found within the pulmonary acini, the typical findings of ACD combined with misalignment of pulmonary veins (MPV). Pathological changes in ACD/MPV resemble a normal fetal lung at the canalicular stage and can therefore also be seen as an arrest of pulmonary development at this stage [3]. Over a period of about 3 months the infant developed normally. The child underwent evaluation for possible lung transplantation. However, starting at the age of 13 weeks, the child developed sudden attacks of oxygen desaturation whilst breathing normally. The attacks were resistant to therapy. At the age of 101 days, the child did not recover from one of these attacks. An autopsy was not performed. The course of ACD/MPV in female siblings of one mother and two different fathers is different from the inheritance pattern in the literature and eliminates a possible recessive pedigree [5]. The first child died during the neonatal period (day 17), the second, reported here in detail, after 101 days of life, both of respiratory distress and permanent pulmonary hypertension (PPHN). When the second child showed similar symptoms after delivery, and after other causes for respiratory distress and PPHN had been ruled out, ACD/MPV was assumed, and subsequently proven. To date, ACD/MPV is a fatal disease [2,6]. Progress in lung transplantation in infants during the past years offers a therapeutic option for patients with ACD/MPV. As demonstrated by our report, prolonged survival using treatment with iNO could serve as a bridge to lung transplantation [1,4]. C. Licht (&) Æ A. Vierzig Æ B. Roth Department of Paediatrics, Children s Hospital, University of Cologne, Joseph-Stelzmann-Strasse 9, 50924 Cologne, Germany E-mail: christoph.licht@uni-koeln.de Tel.: +49-221-4784391 Fax: +49-221-4785835

Spiral CT angiography in an infant with severe hypoplasia of a long segment of the descending aorta

CT angiography, or the spiral CT technique, is a promising minimally invasive method of visualising the arterial vascular system and can be applied in children in whom ultrasound, MRI and/or angiography or cardiac catheterisation cannot be performed, or where an exact diagnosis cannot be made. CT angiography should be considered in special cases as a diagnostic alternative to MRI, ultrasound and angiography. As an example of the possibilities of CT angiography, a case is described in which hypoplasia of the descending aorta was diagnosed and a postoperatively encountered perigraft reaction was demonstrated. Perforation of the blood vessel could be excluded by CT angiography.

DOWN SYNDROME AS A FACTOR INFLUENCING HEMODYNAMIC RESPONSE TO PULMONARY ARTERY BANDING

Abstract. The hemodynamic response to pulmonary artery banding (PAB) in relation to the preoperative pulmonary/systemic vascular resistance (Rp/Rs) ratio and to the timing of surgery, with special regard to Down syndrome, was investigated in 56 nonconsecutive pediatric patients aged 3 days to 6 months (mean 2.5 months) with simple and complex congenital shunt-related cardiac malformations. Among the non-Down patient group (39 patients; mean age 6.9 weeks) there was a good hemodynamic response in all but three cases, irrespective of the preoperative Rp/Rs ratio; these three poor responders had preoperatively normal or nearly normal Rp/Rs ratios (Rp/Rs < 0.3) and were affected postoperatively by lung complications. In the Down patient group (17 patients; mean age 8.2 weeks) the mean preoperative as well as the mean postoperative Rp/Rs ratio was higher than in the non-Down patient group (preoperative Rp/Rs 0.49 versus 0.32; postoperative Rp/Rs 0.31 versus 0.18). There was a good hemodynamic response in all five patients with Down syndrome who had preoperative normal or nearly normal pulmonary vascular resistance ratios (Rp/Rs < 0.3). Among 12 patients with Down syndrome and preoperative increased resistance ratios (Rp/Rs > 0.3) PAB did not cause a reduction in pulmonary vascular resistance (PVR) in five patients (postoperative Rp/Rs 0.49–1.00), all operated on at more than 6 weeks of age. PAB resulted in effective reduction of postoperative Rp/Rs ratios (range 0.10–0.27) in seven patients, six of them younger and one older than 6 weeks at the time of the banding procedure. In conclusion, patients with Down syndrome and shunt-related cardiac malformations (predominantly total atrioventricular canal cases) in general have higher pre- and postoperative Rp/Rs ratios than non-Down children and also have a higher potential for developing pulmonary vascular obstructive disease despite hemodynamically effective PAB. Especially in children with Down syndrome and pathologically high resistance ratios, PAB, if indicated, should be performed as early as possible.

Direct non-medical and indirect costs for families with children with congenital cardiac defects in Germany: a survey from a university centre.

INTRODUCTION Parents of children with congenital cardiac disease suffer from psychological stress and financial burdens. These costs have not yet been quantified. MATERIALS AND METHODS In cooperation with paediatricians, social workers, and parents, a questionnaire was devised to calculate direct non-medical and indirect costs. Direct non-medical costs include all costs not directly related to medical services such as transportation. Indirect costs include lost productivity measured in lost income from wages. Parents were retrospectively queried on costs and refunds incurred during the childs first and sixth year of life. The questionnaire was sent out to 198 families with children born between 1980 and 2000. Costs were adjusted for inflation to the year 2006. Children were stratified into five groups according to the severity of their current health status. RESULTS Fifty-four families responded and could be included into the analysis (27.7%). Depending on severity, total direct non-medical and indirect costs in the first year of life ranged between an average of euro1654 in children with no or mild (remaining) cardiac defects and an average euro2881 in children with clinically significant (residual/remaining) findings. Mean expenses in the sixth year of life were as low as euro562 (no or mild (remaining) cardiac defects) and as high as euro5213 (potentially life-threatening findings). At both points in time, the highest costs were lost income and transportation; and day care/ babysitting for siblings was third. DISCUSSION Families of children with congenital cardiac disease and major sequelae face direct non-medical and indirect costs adding up to euro3000 per year on average. We should consider compensating families from low socioeconomic backgrounds to minimise under-use of non-medical services of assistance for their children.

Efficacy of Pulmonary Artery Banding in the Prevention of Pulmonary Vascular Obstructive Disease

In 57 nonconsecutive pediatric patients (mean age: 2.6 months) with shunt-related pulmonary hypertension, the efficacy of pulmonary artery banding (PAB) was analysed retrospectively with special reference to the development of pulmonary vascular obstructive disease (PVOD). The patients were grouped in accordance to morphology, hemodynamics and incidence of PVOD. Efficacy of PAB was assessed by pulmonary to systemic pressure, flow and resistance ratios obtained by heart catheterization performed pre- and postoperatively. PAB was very effective in 49 out of 57 patients (= 85.9%), in 8 cases a progression or possible progression of PVOD must be suggested. Only Down syndrome can be considered as a predisposing factor for the development of PVOD. Based on our results we conclude that PAB is a high effective procedure in the prevention of PVOD; however, despite the surgical effectiveness of PAB, PVOD can occur. Strict post-PAB examination of pulmonary flow and resistance parameters is recommended and the definite surgical repair should be performed as early as possible.

Left-Side Pulmonary Vein Obstruction After Arterial Switch Operation in Infants with D-Transposition of the Great Arteries

Abstract. We describe two cases of left-side pulmonary vein obstruction observed after the arterial switch operation (Jatene) for D-transposition of the great arteries. This appears to be related to left-sided pulmonary vein obstruction occurring coincidently with D-transposition of the great arteries, rather than a consequence of arterial switch operation.

Hemodynamic and functional consequences of intravascular platelet aggregation in skeletal muscle.

Abstract The effect of ADP-induced platelet aggregation upon blood flow, O 2 -consumption, and performance was investigated in isolated and autoperfused working canine gastrocnemius muscles. During steady state work a mean blood flow of 87.1 ± 33.7 ml × min −1 × 100 g −1 and a mean O 2 -consumption of 7.89 ± 4.03 ml × min −1 × 100 g −1 was found. The average amplitude of muscle contraction was 10.4 ± 4.1 mm. Changes of these parameters due to the ADP-infusion were found to depend upon the number of platelets which remained in the muscles during infusion. On the other hand this so-called platelet loss was significantly correlated with the arterial platelet count. In animals with a low arterial platelet count (below 10 4 /mm 3 ) platelet loss did not exceed 2 × 10 9 cells × min −1 × 100 g −1 . Blood flow as well as venous O 2 -saturation increased during ADP-infusion. In an experiment with a high platelet loss (14.3 × 10 9 cells × min −1 × 100 g −1 ) according to a platelet count of 390 × 10 3 /mm 3 muscle contraction and O 2 -consumption were reduced to approximately 30% of their preinfusion values. These changes, however, only were present during the time of ADP-infusion. After its termination the muscles started to recover immediately reaching the control values within 5 min.

Das sollte Schule machen

Die gesamte Familie im Fokus  Erwachsene, die sich einer schweren Operation unterziehen mussten oder an einer chronischen Krankheit leiden, die die Arbeitsfahigkeit beeintrachtigt, gehen zur „Kur“, Anschlussheilbehandlung, Rehabilitation. Dort werden sie weiter behandelt und lernen, den Heilerfolg aufrechtzuerhalten. Auch fur Kinder und Jugendliche mit chronischen Erkrankungen wie Asthma, Neurodermitis, Adipositas u. a. gibt es Kinder- und Jugendrehabilitationen. Schwere chronische Erkrankungen, schwere operative Eingriffe mit oftmals wochen-, auch monatelangen Aufenthalten in Kinderkliniken bedingen zusatzliche Probleme.

Sporttauglichkeit bei Kindern mit angeborenen Herzfehlern

ZusammenfassungAuch Kinder mit einer angeborenen Herzerkrankung sollten bei sportlichen Aktivitäten einbezogen werden, um einer defizitären psychosozialen Entwicklung vorzubeugen. Die behandelnden Ärzte sollten diesbezüglich auf die Kinder und Eltern einwirken. Dazu soll ein Softwareprogramm, kostenlos verfügbar unter http://www.dgpk.org, beitragen, das es dem behandelnden Kinderkardiologen ohne großen Aufwand ermöglicht, ein individuelles und ausführliches Attest über eine uneingeschränkte Teilnahme oder aber evtl. notwendige Einschränkungen auszustellen.AbstractChildren with congenital heart disease also need leisure and sports activities to prevent deficits in psychosocial development. The treating physician involved should therefore encourage these children and their parents; however, on many occasions a clear statement by the pediatric cardiologist as to whether the child should be restricted in physical activity or not would be of great value. A software tool is presented that can be downloaded for free (http://www.dgpk.org) and enables easy generation of a certificate that can be individualized for the child.