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  • 1
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Besides DiGeorge, velocardiofacial and conotruncal anomaly face syndromes, some of the isolated congenital heart diseases have also been associated with a chromosomal deletion in 22q11. These disease entities, which had originally been considered to have a different genetic background, are now included in the CATCH-22 microdeletion complex. CATCH 22 is an acronym for cardiac defect, abnormal facies, thymic hypoplasia or aplasia and T-cell deficiency, cleft palate, hypoparathyroidism, and hypocalcemia. In the present study, we focused on the complex cardiovascular defects (CCVD) and screened 40 patients for a microdeletion of 22q11 by fluorescence in situ hybridization using the D22S75 DNA probe and for associated CATCH features. The patients were from genetic counseling (n = 15) or fetopathology (n = 3) of the Clinical Genetics Department in Marburg and from the Pediatric Cardiology Department (n = 22) in Mainz. Monosomy 22q11 was detected in 9 cases (= 22.5%). Familial transmission with one mildly affected parent and one affected sib each was proven in two cases. The CCVDs comprised complex conotruncal defects such as tetralogy of Fallot, double outlet right ventricle, transposition of great arteries and truncus arteriosus communis, or anomalies of the derivatives of the branchial arch arteries in association with a ventricular septal defect, including one case of atresia of the ductus arteriosus with pulmonary artery aneurysm and resulting in fetal hydrops. All 13 patients with a deletion of 22q11 showed at least one additional CATCH symptom. Most consistently, facial dysmorphy was apparent (92%), while hypocalcemia, mostly at threshold values, was present in 62% and thymic hypoplasia including borderline low T-lymphocyte numbers was observed in 41%. None of the patients presented with a cleft palate. A high intrafamilial variability in expression was also evident with respect to the CCVD. Our findings indicate that seemingly isolated complex cardiovascular defects associated with a 22q11 microdeletion most probably do not represent a distinct subgroup within the CATCH-22 complex but are syndromal in nature with extracardiac features that are often overlooked.
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  • 2
    ISSN: 1432-1076
    Keywords: Mucopolysaccharidoses ; Mitral regurgitation ; Aortic regurgitation ; Doppler echocardiography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In echocardiographic and necropsy studies nodular thickening of the mitral valve and, less frequently, of the aortic valve has been found in 60%–90% of patients with mucopolysaccharidoses (MPS). Little is known about the haemodynamic consequences of these morphological changes. In this study 84 unselected patients with different enzymatically proven MPS and 84 age and sex matched, healthy persons were studied prospectively by colour Doppler flow mapping. The patients' age ranged from 1 to 47 years (median 8.1 years). Mitral and aortic regurgitation were defined as a holosystolic or holodiastolic jet originating from the valve into the left atrium or the left ventricular outflow tract, respectively, with peak velocities exceeding 2.5 m/s. Of the 84 patients with satisfactory studies, mitral regurgitation was detected in 64.3% and aortic regurgitation in 40.5%, respectively. Regurgitation was severe in 4.8% of mitral valves and 8.3% of aortic valves. The frequency of aortic and/or mitral regurgitation was 75% in all patients, 89% in MPS I, 94% in MPS II, 66% in MPS III, 33% in MPS IV, and 100% in MPS VI. Combined mitral and aortic regurgitation was present in 29% of our patients. None of the control persons showed mitral or aortic regurgitation.
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  • 3
    ISSN: 1432-1238
    Keywords: Fick principle ; Thermodilution ; Cardiac output ; Infants ; Cardiac surgery
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Objective To compare a system that continuously monitors cardiac output by the Fick principle with measurements by the thermodilution technique in pediatric patients. Design Prospective direct comparison of the above two techniques. Setting Pediatric intensive care unit of a university hospital. Patients 25 infants and children, aged 1 week to 17 years (median 10 months), who had undergone open heart surgery were studied. Only patients without an endotracheal tube leak and without a residual shunt were included. Methods The system based on the Fick principle uses measurements of oxygen consumption taken by a metabolic monitor and of arterial and mixed venous oxygen saturation taken by pulse- and fiberoptic oximetry to calculate cardiac output every 20 s. Interventions In every patient one pair of measurements was taken. Continuous Fick and thermodilution cardiac output measurements were performed simultaneously, with the examiners remaining ignorant of the results of the other method. Results Cardiac output measurements ranged from 0.21 to 4.55 l/min. A good correlation coefficient was found:r 2=0.98;P〈0.001; SEE=0.14 l/min. The bias is absolute values and in percent of average cardiac output was −0.05 l/min or −4.4% with a precision of 0.32 l/ min or 21.3% at 2 SD, respectively. The difference was most marked in a neonate with low cardiac output. Conclusion Continuous measurement of cardiac output by the Fick principle offers a convenient method for the hemodynamic monitoring of unstable infants and children.
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  • 4
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
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  • 5
    ISSN: 1432-1076
    Keywords: Newborn ; Cardiac surgery ; Pulmonary hypertension ; Prostacyclin ; Nitric oxide
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In a 3-week-old male newborn persistent suprasystemic pulmonary hypertension developed after surgical valvulotomy for a critical aortic valve stenosis. Because of a residual transvalvular pressure gradient of 35 mm Hg and postoperative left as well as right ventricular dysfunction, treatment with inhaled nitric oxide (NO) and intravenously infused prostacyclin (PGI2) was attempted. Low-dose inhaled NO and low dose PGI2 corrected severe pulmonary hypertension and led to an increase in cardiac output. Treatment with NO but not PGI2 was accompanied by a rise in PaO2 and systemic blood pressure. Interruption of NO administration led to a rapid increase in pulmonary arterial pressure to suprasystemic levels. With continued i.v. PGI2 and decreasing concentrations of NO, severe pulmonary hypertension resolved after a few days suggesting that a transient endothelial dysfunction was partially responsible for pulmonary vasoconstriction. NO inhalation appears to be an effective new tool in the treatment of severe pulmonary hypertension following cardiac surgery.
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  • 6
    ISSN: 1432-1076
    Keywords: Key words Mucopolysaccharidoses ; Mitral regurgitation ; Aortic ; regurgitation ; Doppler ; echocardiography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In echocardiographic and necropsy studies nodular thickening of the mitral valve and, less frequently, of the aortic valve has been found in 60%–90% of patients with mucopolysaccharidoses (MPS). Little is known about the haemodynamic consequences of these morphological changes. In this study 84 unselected patients with different enzymatically proven MPS and 84 age and sex matched, healthy persons were studied prospectively by colour Doppler flow mapping. The patients' age ranged from 1 to 47 years (median 8.1 years). Mitral and aortic regurgitation were defined as a holosystolic or holodiastolic jet originating from the valve into the left atrium or the left ventricular outflow tract, respectively, with peak velocities exceeding 2.5 m/s. Of the 84 patients with satisfactory studies, mitral regurgitation was detected in 64.3% and aortic regurgitation in 40.5%, respectively. Regurgitation was severe in 4.8% of mitral valves and 8.3% of aortic valves. The frequency of aortic and/or mitral regurgitation was 75% in all patients, 89% in MPS I, 94% in MPS II, 66% in MPS III, 33% in MPS IV, and 100% in MPS VI. Combined mitral and aortic regurgitation was present in 29% of our patients. None of the control persons showed mitral or aortic regurgitation. Conclusion Aortic and mitral regurgitation are more frequent in patients with MPS than previously thought and that therefore these patients should have regular colour Doppler flow mapping and antibiotic prophylaxis when required.
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  • 7
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Epikarde Elektroden ; Steroideluierende Elektroden ; Autocapture ; Frühgeborene ; Säugling ; Key words Epicardial pacing ; Steroid eluting electrodes ; Autocapture ; Infant
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary A small pulse-generator is important in prematures and young infants. Objectives: Analysis of our experience with the smallest currently available pacemaker in the pediatric age group. Patients and methods: We report about the pacemaker therapy in two premature babies with symptomatic complete congenital AV-block and about an 8 year old boy with congenital AV-block and palliation of complex congenital heart disease by total cavo pulmonary connection. The currently smallest pulse generator was implanted for the first time with a steroid eluting epicardial bipolar lead. Due to the ’autocapture algorithm’ a smaller battery can be used with the same life span as a normal sized pulse generator. Results: A stable capture threshold with a high amplitude of the evoked response signal and regular function of the ’autocapture’ algorithm was documented during a followup of up to 1 year. This algorithm has been used so far only with endocardial leads. Conclusion: This new combination seems promising for pacemaker therapy in small children.
    Notes: Zusammenfassung Fragestellung: Für Säuglinge und Frühgeborene ist eine geringe Größe des Schrittmacheraggregats besonders wichtig. Unsere bisherigen Erfahrungen mit dem kleinsten, z.Z. verfügbaren Pulsgenerator bei Kindern wurden analysiert und hier vorgestellt. Methode: Bei 2 Frühgeborenen mit symptomatischem kompletten AV-Block und einem 8jährigen Patienten mit kongenitalem AV-Block bei komplexen Vitium und Palliation mittels totaler kavopulmonaler Anastomose wurde der kleinste z.Z. verfügbare Pulsgenerator erstmals mit bipolaren, epikardialen steroideluierenden Elektroden implantiert. Dieser Schrittmacher arbeitet durch den Autocapture-Algorithmus besonders energiesparend, so daß trotz geringerer Batteriekapazität eine Laufzeit wie bei einem normal großen Aggregat erzielt werden kann. Ergebnisse: Bei einer niedrigen Reizschwelle und einer hohen Amplitude des evozierten Potentials fand sich eine normale Funktion des Autocapture-Algorithmus des Schrittmachers bei einer Nachbeobachtung bis zu 1 Jahr. Ein solches Aggregat wurde bislang nur mit endokardialen Elektroden eingesetzt. Schlußfolgerung: Diese Schrittmacher-Elektroden-Kombination erscheint als neues Therapiekonzept insbesondere für Säuglinge gut geeignet zu sein.
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  • 8
    ISSN: 1460-9592
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Pulmonary air leaks are one of the most common problems in patients with the adult respiratory distress syndrome, ARDS. We report what we believe to be the first case in which unilateral high-frequency ventilation combined with contralateral conventional positive pressure ventilation has been used successfully to manage severe air leak in an infant with ARDS.
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  • 9
    ISSN: 1432-1238
    Keywords: Key words Fick principle ; Thermodilution ; Cardiac output ; Infants ; Cardiac surgery
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract   Objective:To compare a system that continuously monitors cardiac output by the Fick principle with measurements by the thermodilution technique in pediatric patients. Design: Prospective direct comparison of the above two techniques. Setting: Pediatric intensive care unit of a university hospital. Patients: 25 infants and children, aged 1 week to 17 years (median 10 months), who had undergone open heart surgery were studied. Only patients without an endotracheal tube leak and without a residual shunt were included. Methods: The system based on the Fick principle uses measurements of oxygen consumption taken by a metabolic monitor and of arterial and mixed venous oxygen saturation taken by pulse- and fiberoptic oximetry to calculate cardiac output every 20 s. Interventions: In every patient one pair of measurements was taken. Continuous Fick and thermodilution cardiac output measurements were performed simultaneously, with the examiners remaining ignorant of the results of the other method. Results: Cardiac output measurements ranged from 0.21 to 4.55 l/min. A good correlation coefficient was found: r 2=0.98; P〈0.001; SEE=0.14 l/min. The bias is absolute values and in percent of average cardiac output was −0.05 l/min or −4.4% with a precision of 0.32 l/min or 21.3% at 2 SD, respectively. The difference was most marked in a neonate with low cardiac output. Conclusion: Continuous measurement of cardiac output by the Fick principle offers a convenient method for the hemodynamic monitoring of unstable infants and children.
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