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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Pacing and clinical electrophysiology 23 (2000), S. 0 
    ISSN: 1540-8159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The results of epicardial pacing in infants with isolated congenital complete atrioventricular block (CAVB) are reported. Thirty-four patients, aged 1 day to 20 months (22 patients 〈 1 month), were paced between 1988 and 1998. Thirty had bradycardia 〈 50 beats/min with symptoms in 12 patients, and 4 patients were paced because of associated ventricular ectopy or prolonged QT interval. In thirty cases, the electrodes were implanted through a left thoracotomy and connected to an abdominal generator; in four, the sub-xyphoid approach was preferred. Twenty-two children had dual chamber units. There was no operative death, but three patients died later of cardiomyopathy. Seven infants were reoperated for electrode displacement, infection, exit block, and pacemaker sensitivity. Chronic ventricular thresholds ranged from 0.3 to 2 V except in one case (4 V) and proper atrial sensing was lost in two cases. All children are doing well and the generator has lasted at least 5 years in 16 cases. In conclusion, epicardial pacing in infants with CAVB can be done with satisfactory results. There was no mortality in relation with pacing and thresholds have improved with the use of steroid-eluting electrodes. The deep location of the generator prevents cutaneous erosion and allows implantation of large units with a longer life duration.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A case of transcatheter occlusion of a modified Blalock-Taussig (BT) shunt with a detachable balloon is described. A three month old boy with pulmonary atresia with intact ventricular septum had a repair consisting in valvotomy and a modified BT. This palliative aorticopulmonary shunt created congestive heart failure. As an alternative to surgery, a detachable balloon was used to occlude the BT shunt.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1084
    Keywords: Key words: Contrast media ; Iopentol ; Iopromide ; Pediatric angiocardiography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary. Iopentol (Imagopaque®, Nycomed Imaging AS, Oslo, Norway) 300 mg I/ml was compared with iopromide (Ultravist®, Schering AG, Berlin, Germany) 300 mg I/ml in pediatric angiocardiography in 97 children (48 and 49 patients, mean age 2.8 and 4.1 years in the respective contrast medium groups). The volume injected was usually 4–6 ml/kg b. w. ECG, blood pressure, heart rate, adverse events and efficacy were evaluated. Five patients in the iopentol group and four in the iopromide group reported adverse events. This difference was not statistically significant. One adverse event in each of the two groups was considered as possibly related to the contrast medium. Only small and transient changes in heart rate, blood pressure and ECG parameters were observed. No difference between the two contrast media was found for efficacy. It can be concluded that iopentol is well suited for angiocardiographic examinations in children.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Intensive care medicine 2 (1976), S. 1-1 
    ISSN: 1432-1238
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Intensive care medicine 15 (1989), S. S76 
    ISSN: 1432-1238
    Keywords: Organ transplantation ; Kidney transplantation ; Heart transplantation ; Heart and lung transplantation ; Liver transplantation ; Small intestine transplantation ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Organ transplantations have opened a new field in medicine and particularly in pediatrics. The kidney was the first organ to be regularly transplanted and there are now more than 2000 children who have received a kidney graft. Cadaver kidneys or living-related donor (LRD) kidneys can be used since an adult kidney may be grafted in a young child. Cadaver graft survival exceeds 85% at 1 year in recent single centre reports and patient survival is above 95%, the results being even better with LRD. Some complications may be observed in the long term, such as bone osteonecrosis, hypertension or infections. Rehabilitation is generally remarkable and growth which remained abnormal in 1/3 of cases under conventional treatment seems to improve markedly with cyclosporin. In the future, the development of kidney transplantation could lead hopefully to a drastic decrease in the number of children on dialysis. Liver transplantation is now performed in children with biliary atresia and metabolic diseases. There is no age limit for grafting a liver, the only problem being to find a pediatric cadaver donor of similar size. It is also possible to use an adult liver after hepatectomy. There were 170 liver grafts recorded in children in Europe in December 1986, and the long-term survival thanks to cyclosporin is exceeding 80% in some units. Thanks to cyclosporin, programs of cardiac transplantation for children are rapidly developing. The main indications are gross-congenital cardiac malformations or severe functional abnormality not compatible with life. Newborns have been grafted with success. The only problem is also to find a suitable pediatric donor. At the end of 1984, there were 88 cadaver transplantations reported in children, and this number is growing rapidly. Survival is estimated above 70% at 1 year. Indications for heart and lung transplantation are more numerous than for the heart alone, including severe cardiac malformations with abnormality of pulmonary circulation and a number of pulmonary diseases. A close similarity in the size of donor and recipient is mandatory. The immediate results are generally good, but the final outcome remains under evaluation. First investigated in animals, small intestine transplantation has been added recently to the list of possible organ transplantations in children. Indications are extensive intestinal resections, or malformations. The initial results are encouraging, but the long-term outcome remains under evaluation.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 151 (1992), S. S59 
    ISSN: 1432-1076
    Keywords: Heart transplantation ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract From 1987 to 1991, heart transplantation was undertaken in 49 infants and children with either end-stage cardiomyopathies (28 patients) or severe congenital heart disease (21 patients including 16 having already been surgically but unsuccessfully treated). Their age ranged from 13 days to 15 years (mean=4.5±4.2 years; median=2.5 years). There were 12 early and 7 late deaths (overall mortality=38%), mainly due to graft dysfunction, acute or chronic rejection, and infectious complications, mostly viral. Optimal criteria in selecting both donors and recipients are crucial to reduce early mortality and should never be transgressed despite the critical shortage of organs. The actuarial probability of survival was 64% at 1 year and 57% at 5 years. Our 30 mid-term survivors (62%) were submitted to a close follow up programme which includes endomyocardial biopsies, even in the very young, since non invasive criteria failed to mark every rejection episode. Maintenance therapy was always steroid-free to start with (cyclosporin + azathioprine) but in almost one half of our oldest survivors, it failed to avoid rejection and we had to add lowdose oral steroids for at least several months. Epstein-Barr virus related lymphoproliferations occurred in four patients, two of whom died and two recovered with specific therapy. Renal function was closely monitored: tubular and interstitial lesions were found on renal biopsies and were associated with moderate functional changes. The quality of life of the children who survived heart transplantation was considered as near normal in a little more than one half of the cases but many issues (late coronary disease, drug toxicity, long-term compliance to follow up and therapy) remain significant concerns for the future.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1076
    Keywords: Key words Chromosome 22q11.2 microdeletions ; Congenital heart disease ; Conotruncal heart defect ; DiGeorge anomaly
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Conotruncal malformations account for about 50% of congenital heart defects diagnosed in newborns. We studied prospectively 104 patients admitted in our neonatal intensive care unit for conotruncal defects by fluorescence in situ hybridization to estimate the prevalence of the interstitial deletion in this category of congenital heart disease. Cardiac phenotypes were: truncus arteriosus (17), interrupted aortic arch (18), tetralogy of Fallot with or without pulmonary valve atresia (55), tetralogy of Fallot with absent pulmonary valves (5), ventricular septal defect with malalignment of the conal septum (9). We discovered a microdeletion 22q11 at loci D22S39 or D22S398 in 50 newborns (48%). The prevalence of this microdeletion in different groups of conotruncal defects was: truncus arteriosus 7/17, interrupted aortic arch 16/18, tetralogy of Fallot 19/55, absent pulmonary valves 2/5, and ventricular septal defect 6/9 respectively. Only two patients without any clinical or biological feature of the so called CATCH22 syndrome exhibited the deletion. Parental studies confirmed that the deletion occurred de novo in 47/50 cases (three parental microdeletions). On the other hand, recurrence of conotruncal heart defects in families of “undeleted probands” was higher than expected (13%). Conclusion In 50/104 newborns with conotruncal defects, an interstitial deletion 22q11 was found. Fluorescence in Situ Hybridization should be performed in newborn infants with conotruncal defect and at least one additional manifestation of the CATCH22 phenotype.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1076
    Keywords: Key words Williams syndrome ; Coronary artery ; Myocardial infarction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report a 3-year-old child with Williams syndrome in whom the first vascular feature of the syndrome was a myocardial infarction related to the occlusion of the left main coronary artery trunk. This coronary artery occlusion was not associated with supravalvular aortic stenosis. Conclusion This report emphazises that acute vascular events related to systemic artery anomalies may reveal Williams syndrome.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 412 (1987), S. 27-29 
    ISSN: 1432-2307
    Keywords: Restrictive cardiomyopathy ; Endomyocardial fibrosis ; Infiltrative changes of the myocardium ; Intracellular deposits
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Restrictive cardiomyopathy is usually related to fibrosis of the endocardium or to an infiltrative disorder. However, in few cases, it can be due to isolated pathology of the myocytes but such alterations are not well characterized. This paper reports the disease in two 7 year old patients. There was severe venous congestion and catheterisation revealed increased end diastolic pressure in the ventricles. Both pericardial and myocardial biopsies were performed, as the clinical and haemodynamic data were indistinguishable from constrictive pericarditis. The structure of the pericardium was normal. The endocardium was not thickened. The interstitium of the myocardial tissue was not increased. Electron microscopic examination revealed intracellular masses of disorganized myofilaments. These large deposits may have produced decrease compliance of the myocytes and of the ventricular walls.
    Type of Medium: Electronic Resource
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