Combined Klinefelter-Down syndrome or XXYY syndrome?
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Cell Nucleus↗
Biomedical subjects
H Singer
Publications and source records attributed to H Singer.
[Coronary vessel anomalies and persisting myocardial sinusoids in pulmonary atresia with intact ventricular septum].
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Cardiac Catheterization↗
The syndrome of absent pulmonary valve and ventricular septal defect--anatomical features and embryological implications.
Four cases of absent pulmonary valve in combination with ventricular septal defect are reported. In this syndrome hypo- and dysplasia of the pulmonary valve is constantly associated with a big ventricular septal defect, formation of a huge pulmonary artery aneurysm and absence of the ductus arteriosus. Presence or absence of a right ventricular outflow tract obstruction is the criteria for classification into two forms. Absence of the pulmonary valve, right ventricular outflow tract obstruction and a malalignment-type ventricular septal defect produced by a conotruncal malseptation process represent the primary complex of malformations. Consecutive intrauterine cardiac failure is most probably prevented by prenatal closure of the ductus arteriosus. Pulmonary artery aneurysm and also dilatation of the right ventricular outflow tract as well as a whole lot of other coexisting deformities can be explained by a cascade of hemodynamical sequelae started by this ductus closure in utero. An embryological scheme explaining the genesis of this syndrome is derived from a morphological analysis of the constituting incoherent-appearing anatomical features.
Angiocardiography↗
[Experiences with capnometry in pediatric intensive care].
We investigated the correlation between arterial and end-tidal pCO2 in 17 children during intensive care. The indications for using capnometry were noninvasive monitoring of ventilation after cardiac surgery or during complicated course of illness or for control of intended hyperventilation. We found a good correlation between arterial and end-tidal pCO2 in the overall patient population (r = 0.82), but the relationship varied widely from patient to patient. There was only a weak correlation (r = 0.44) in pCO2 over 45 mm Hg. Hyperventilation could be identified in 64.2%, whereas hypoventilation was correctly indicated in only 23%.
Carbon Dioxide↗
[Doppler sonographic flow measurement in the anterior cerebral artery and celiac trunk in infants with coarctation syndrome in comparison with healthy infants].
15 infants (gestational age 47 +/- 11 weeks; weight 3180 +/- 1082 g) with angiocardiographically proven coarctation of the aorta and 53 healthy infants (gestational age 39 +/- 8 weeks; weight 2830 +/- 1418 g) were investigated by echocardiography and pulsed doppler sonography. In all children pulsed doppler recordings were obtained in the anterior cerebral artery (prestenotic reference vessel) and the truncus coeliacus (poststenotic reference vessel). Nine variables were measured from each doppler signal: The maximal systolic velocity, the endsystolic and enddiastolic velocity, the pulsatility-index, the acceleration slope (peak rate of acceleration), the deceleration slope (peak rate of deceleration), the antegrade flow time and the acceleration and deceleration time. The healthy infants showed a pulsatile flow profile in the anterior cerebral artery and the truncus coeliacus. In normal subjects the maximal systolic velocity and the acceleration slope in the truncus coeliacus were always higher than in the anterior cerebral artery. There was no difference in the endsystolic and enddiastolic velocity, the pulsatility-index, the flow times and the deceleration slope. In children with coarctation of the aorta a decreased and nonpulsatile flow in the truncus coeliacus could be shown. The following variables were significantly lower than in the healthy control group: The maximal systolic velocity, the pulsatility-index, the acceleration and deceleration slope and the antegrade flow time and acceleration time. In the anterior cerebral arteries of children with coarctation the maximal systolic and enddiastolic velocity were significantly higher than in the healthy control group. Pulsed doppler recordings were obtained in 9 patients after surgical correction: The maximal systolic and endsystolic velocity, the pulsatility-index, the acceleration and deceleration slope in the truncus coeliacus increased significantly although these values were lower than in the healthy control group. The flow parameters in the anterior cerebral arteries postoperatively showed no significant difference from the preoperative values. Pulsed dopplersonography of the flow in the anterior cerebral artery and the truncus coeliacus is a good method for diagnosis of coarctation of the aorta and postoperative controls.
Aortic Coarctation↗
[Neonatal tricuspid insufficiency--a contribution to the differential diagnosis of the cyanotic newborn infant].
A cyanotic newborn is described to illustrate and discuss the rare transient tricuspid insufficiency together with functional pulmonary atresia. Etiologically the manifold causes of perinatal asphyxia are to be taken into consideration. The clinical appearance corresponds to type A of transient myocardial dysfunction according to the classification of Rowe together with respiratory distress syndrome and heart failure. Pathogenetically the disease is based on the different histological consequences of myocardial ischaemia of the right ventricle in connection with a delayed fall of the pulmonary arterial resistance. Fetal connections lead to a pronounced right-to-left-shunt. The diagnosis is primarily established by the way of clinical and echocardiographic investigation and pulsed doppler examination. The most important differential diagnosis are in Ebstein-anomaly, obstruction and atresia of the right ventricular outflow tract. Therapy is conservative. Its aim is to improve myocardial performance and to lower right ventricular afterload. Outcome of patients with neonatal tricuspid insufficiency is uncertain and depends on the severity of myocardial ischaemia. In our patient the course has been uneventful after initial difficulties.
Cardiac Catheterization↗
[Ductus aneurysm as a rare cause of inspiratory stridor in the newborn infant].
A paralysis of the left vocal cord was seen by laryngoscopy in a 3-days-old boy with inspiratory stridor. A ductus aneurysm was established by angiocardiography, which was believed to be the cause of the vocal cord paralysis. Within 8 days after the angiocardiography the aneurysm became smaller and the stridor disappeared. Twelfth months later the control-angiocardiography showed the total obliteration of the ductal aneurysm.
Aneurysm↗
[Doppler sonographic flow parameter of the anterior cerebral artery in patent ductus arteriosus of the newborn infant compared to a healthy control sample].
33 premature infants (age: 32 +/- 3 weeks; birth weight 1,268 +/- 535 gs) with the clinical signs of patent ductus arteriosus Botalli (PDA) and a control group of 96 healthy infants (age: 37 +/- 4 weeks; birth weight 2 348 +/- 944 gs) were investigated. Pulsed doppler recordings were obtained in the anterior cerebral arteries (ACA) and compared with the flow pattern in the truncus coeliacus (TC). In all children the maximal systolic velocity (Vs), the endsystolic (Ves) and the enddiastolic velocity (Ved) and the pulsatility-index (PI) were measured. The 96 healthy premature born infants showed the following velocities: Vs: 41 +/- 12 cm X sec-1; Ves: 19 +/- 7 cm X sec-1; Ved: 10 +/- 4 cm X sec-1. The pulsatility-index was 0.74 +/- 0.08. In children with PDA all velocities were significantly lower than in the healthy control group: Vs: 31 +/- 10 cm X sec-1; Ves: 7 +/- 6 cm X sec-1; Ved: -1 +/- 5 cm X sec-1. Ved was more decreased than Vs resulting in a significant increase in PI (1.04 +/- 0.14). 22 infants with surgically proven large PDA (age: 31 +/- 3 weeks; birth weight: 1,160 +/- 467 gs) showed significant lower velocities (Vs: 34 +/- 8 cm X sec-1; Ves: 4 +/- 4 cm X sec-1; Ved: -4 +/- 4 cm X sec-1) in comparison with the healthy control group and the 11 children with small PDA (age: 33 +/- 4 weeks; birth weight: 1,494 +/- 621 gs).(ABSTRACT TRUNCATED AT 250 WORDS)
Birth Weight↗
[Problem of fetal arrhythmias--a case report].
A fetal congenital complete AV-block was detected in the 30th week of gestation. After close sonographic and cardiotocographic follow-up examinations premature labor occurred in the 37th week of gestation. The interpretation of the fetal heart rate pattern was difficult. The bradycardia was accompanied by late decelerations (dip II), only different from familiar hypoxia-patterns by the bradycardia. Caesarean section therefore was immediately done. A partial abruptio placentae was found as causing hypoxia. The child developed well after insertion of a temporary transvenous pacemaker. The AV-block is still persisting. At the age of three months the child fell ill of an acute cutaneous lupus erythematosus. The symptoms disappeared after unspecific treatment. Antinuclear antibodies pathognomonic for lupus erythematosus were traceable in the symptomless mother. There are other observations of motherly autoantibodies passing the placental barrier impairing the fetal heart conduction.
Adult↗