Das zyanotische Kind mit kardialer Erkrankung im Notfalldienst

Zusammenfassung: Im Gegensatz zu Erwachsenen mit vorwiegend pulmonalen Erkrankungen wie der chronisch obstruktiven Lungenerkrankung, entsteht bei Kindern eine zentrale Zyanose meist durch angeborene Herzfehler. Zyanotische Herzfehler kommen mit einer Häufigkeit von ca.13 pro 10.000Lebendgeburten vor. Dadurch, dass heutzutage auch komplexe Herzfehler erfolgreich palliiert werden, steigt die Wahrscheinlichkeit stetig an, im Rettungsdienst Kindern mit einer zentralen Zyanose zu begegnen. Ursachen für das untersättigte Blut in den großen Arterien können eine Minderperfusion der Lungen oder eine vermehrte Durchmischung von arteriellen mit venösem Blut über einen Rechts-Links-Shunt sein. Der beste Indikator einer zentralen Zyanose ist die Zunge, da sie reichlich vaskularisiert und frei von Pigmenten ist. Je nach Altersklasse ist eher eine primäre Volumentherapie oder eine großzügige Sauerstoffapplikation essenziell, oder aber auch keine Notfalltherapie notwendig. Nach der präklinischen Versorgung ist eine stationäre Einweisung unabdingbar, bevorzugt in ein Zentrum mit der Option der kinderintensivmedizinischen oder kinderkardiologischen Betreuun

Impact of right ventricular size on ECG after percutaneous closure of atrial septal defect with Amplatzer Septal Occluder.

To assess ECG changes after percutaneous atrial septal defect (ASD) closure in children with significant left-to-right shunt. Analysis of data of 36 consecutive children with an ASD who had successful percutaneous ASD closure with an Amplatzer Septal Occluder. Assessment comprised echocardiography and ECG the day before and after the procedure and at 1, 6 and 12 months follow-up. The median age (interquartile range) of children was 7.3 (5.3) years. On the day after the procedure the end diastolic diameter of the right ventricle showed already a diminution (34 (12) mm/m2 before intervention vs. 32 (12) mm/m2). ECG changes were first observed at 1 month follow-up (PR interval before intervention 139 (20) ms vs. 132 (20) ms; QRS duration 88 (18) ms vs. 82 (19) ms) and at 6 months follow-up (QRS axis 77 degrees (33) before intervention vs. 72 degrees (53)). With the exception of the QRS duration, ECG intervals and axis were in a normal range in all patients before the procedure. Median QRS duration normalised at 1 year follow-up (83 (8) ms). After transcatheter ASD closure, decrease in right ventricular size began rapidly and was followed by reduction of the QRS duration and PR interval within weeks. Shifting to the left of the QRS axis was observed within 6 months follow-up. This study showed that ECG changes due to right ventricular volume overload can regress and normalise after percutaneous ASD closure in children

The Fate of Children with Microdeletion 22q11.2 Syndrome and Congenital Heart Defect: Clinical Course and Cardiac Outcome

Background: This study aimed to evaluate the cardiac outcome for children with microdeletion 22q11.2 and congenital heart defect (CHD). Methods: A total of 49 consecutive children with 22q11.2 and CHD were retrospectively identified. The CHD consisted of tetralogy of Fallot and variances (n = 22), interrupted aortic arch (n = 10), ventricular septal defect (n = 8), truncus arteriosus (n = 6), and double aortic arch (n = 1). Extracardiac anomalies were present in 46 of 47 children. Results: The median follow-up time was 8.5 years (range, 3 months to 23.5 years). Cardiac surgical repair was performed for 35 children, whereas 5 had palliative surgery, and 9 never underwent cardiac surgery. The median age at repair was 7.5 months (range, 2 days to 5 years). The mean hospital stay was 35 days (range, 7-204 days), and the intensive care unit stay was 15 days (range, 3-194 days). Significant postoperative complications occurred for 26 children (74%), and surgery for extracardiac malformations was required for 21 patients (43%). The overall mortality rate was 22% (11/49), with 1-year survival for 86% and 5-year survival for 80% of the patients. A total of 27 cardiac reinterventions were performed for 16 patients (46%) including 15 reoperations and 12 interventional catheterizations. Residual cardiac findings were present in 25 patients (71%) at the end of the follow-up period. Conclusions: Children with microdeletion 22q11.2 and CHD are at high risk for mortality and morbidity, as determined by both the severity of the cardiac lesions and the extracardiac anomalies associated with the microdeletio

Cardiac output measurement in children: comparison of Aesculon® cardiac output monitor and thermodilution

Background We compared cardiac output (CO) measurements by the non-invasive electrical velocimetry (Aesculon®) monitor with the pulmonary artery catheter (PAC) thermodilution method in children. Methods CO values using the Aesculon® monitor and PAC thermodilution were simultaneously recorded during cardiac catheterization in children. Measurements were performed under general anaesthesia. To compare, three consecutive measurements for each patient within 3 min were obtained. The means of the three values were compared using simple regression and Bland-Altman analysis. Data were presented as mean (sd). A mean percentage of <30% was defined to indicate clinical useful reliability of the Aesculon® monitor. Results A total of 50 patients with a median (range) age of 7.5 (0.5-16.5) yr were enrolled in the study. Mean CO values were 3.7 (1.5) litre min−1 (PAC thermodilution) and 3.1 (1.7) litre min−1 (Aesculon® monitor). Analysis for CO measurement showed a good correlation between the two methods (r=0.894; P<0.0001). The bias between the two methods was 0.66 litre min−1 with a precision of 1.49 litre min−1. The mean percentage error for CO measurements was 48.9% for the Aesculon® monitor when compared with PAC thermodilution. Conclusions Electrical velocimetry using the Aesculon® monitor did not provide reliable CO values when compared with PAC thermodilution. Whether the Aesculon® monitor can be used as a CO trend monitor has to be assessed by further investigations in patients with changing haemodynamic

The fate of children with microdeletion 22q11.2 syndrome and congenital heart defect: clinical course and cardiac outcome

BACKGROUND: This study aimed to evaluate the cardiac outcome for children with microdeletion 22q11.2 and congenital heart defect (CHD). METHODS: A total of 49 consecutive children with 22q11.2 and CHD were retrospectively identified. The CHD consisted of tetralogy of Fallot and variances (n = 22), interrupted aortic arch (n = 10), ventricular septal defect (n = 8), truncus arteriosus (n = 6), and double aortic arch (n = 1). Extracardiac anomalies were present in 46 of 47 children. RESULTS: The median follow-up time was 8.5 years (range, 3 months to 23.5 years). Cardiac surgical repair was performed for 35 children, whereas 5 had palliative surgery, and 9 never underwent cardiac surgery. The median age at repair was 7.5 months (range, 2 days to 5 years). The mean hospital stay was 35 days (range, 7-204 days), and the intensive care unit stay was 15 days (range, 3-194 days). Significant postoperative complications occurred for 26 children (74%), and surgery for extracardiac malformations was required for 21 patients (43%). The overall mortality rate was 22% (11/49), with 1-year survival for 86% and 5-year survival for 80% of the patients. A total of 27 cardiac reinterventions were performed for 16 patients (46%) including 15 reoperations and 12 interventional catheterizations. Residual cardiac findings were present in 25 patients (71%) at the end of the follow-up period. CONCLUSIONS: Children with microdeletion 22q11.2 and CHD are at high risk for mortality and morbidity, as determined by both the severity of the cardiac lesions and the extracardiac anomalies associated with the microdeletion

The phenomenology of the mathematics classroom

This paper describes the mathematics classroom from the perspective of social phenomenology. Here the classroom is seen as an environment of signs, comprising things and people, which impinge on the reality of the individual child. The paper introduces a framework through which mathematical work is seen as taking place in the imagined world through the filter of the world in immediate perception. This provides an approach to structuring evolving mathematical understanding. It is suggested that mathematical ideas are contained and shaped by the child's personal phenomenology, which evolves through time. Further, I argue these ideas are never encountered directly but rather are met through a circular hermeneutic process of reconciling expectation with experience. © 1996 Kluwer Academic Publishers

Pulse oximetry screening for congenital heart defects in Switzerland: most but not all maternity units screen their neonate

QUESTIONS: In Switzerland, the screening of neonates for congenital heart defects (CHD) by using pulse oximetry (PO) on the first day of life was recommended in 2005. We assessed the current practice of Swiss maternity units regarding PO screening to define the actual screening rate in Switzerland and to detect possible difficulties in the implementation of this screening. METHODS: This descriptive study was conducted by means of a standardized questionnaire that was sent to all Swiss maternity units. RESULTS: The response rate was 100%. 76% of the units perform regular PO screening and, on the basis of the number of births for 2007, this means that 85% of newborns in Switzerland undergo screening. Although units which do not perform PO screening include every category of maternity unit, the screening is performed to a significantly lower extent in birthing centres than in hospitals (p 1000 births per year). The main reasons for not performing PO screening are financial factors and the belief that clinical signs are sufficient for the detection of CHD. CONCLUSIONS: Greater effort is needed to implement a nationwide PO screening program for CHD. Special attention should be paid to birthing centres given their limited capacity to treat newborns with critical CHD

Experience with implantable cardioverter-defibrillator therapy in grown-ups with congenital heart disease

Implantable cardioverter-defibrillators (ICD) are increasingly implanted for primary or secondary prevention of sudden death in young patients with congenital heart disease, cardiomyopathies, or channelopathies. Although major advances in ICD technology and implant techniques have facilitated ICD therapy in young patients, complications such as lead failures, inappropriate shocks, system infections, and negative psychosocial impacts are of concern. The various underlying cardiovascular disease states and a lack of standardized ICD protocols for young patients often necessitate individualized implant techniques, ICD programming, and follow-up. Young ICD patients need a thorough follow-up to ensure adequate therapy, and psychosocial problems have to be addressed

Myocarditis and coronary dilatation in the 1st week of life: neonatal incomplete Kawasaki disease?

Acute heart failure in the early neonatal period is rare. Normally it is due to asphyxia, severe septicaemia, a congenital heart malformation or a viral myocarditis. Kawasaki disease (KD) as a cause of an neonatal myocarditis is not an established diagnosis. KD is a vasculitis of still unknown origin occurring predominantly in infants and preschool children. KD before the age of 3 months is rare. There are only few reports about KD in the 1st month. We present a newborn who showed the cardiac symptoms of KD in the 1st week of life with coronary dilatation and myocarditis. CONCLUSION: The diagnosis of incomplete KD should be considered not only in infants but also in newborns with signs of myocarditis and coronary abnormalities. Therapy with gammaglobulins may prevent the sequelae of coronary involvement