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  • 1
    ISSN: 1432-1971
    Keywords: Key words: Bland–White–Garland syndrome — Stenotic origin left coronary artery — Mammary artery grafting — Stent implantation — Coil occlusion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. A 16-year-old boy with anomalous origin of the left coronary artery from the pulmonary artery, Bland–White–Garland syndrome, underwent a mammary artery bypass grafting to the left coronary artery (LCA) together with closure of the stenosed origin of the left coronary artery. A residual LCA to pulmonary artery fistula and stenosis of the shunt at the implantation site developed that resulted in a dominant perfusion of the LCA from the right coronary artery. Interventional catheterization was performed with stenting of the LCA mammary artery anastomosis and subsequent coil occlusion of the residual fistula. After this intervention the LCA was exclusively perfused by the mammarian bypass with no residual leak to the pulmonary artery.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1971
    Keywords: Key words: Pulmonary embolism — Fontan operation — Thrombolysis — Tissue plasminogen activator
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. We report the case of a 3-year-old girl who presented with near-lethal pulmonary thrombembolism 3 weeks after an uneventful Fontan operation. Complete occlusion of the left lower lobe pulmonary artery had occurred together with a cerebral infarction. Recombinant tissue plasminogen activator (rt-Pa) was used for thrombolysis because of its short half-life and its clot-selective properties. To further minimize the systemic effects of rt-PA, local catheter-directed lysis was performed. A prolonged course of low-dose rt-PA therapy achieved complete lysis without side effects.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric cardiology 18 (1997), S. 222 -225 
    ISSN: 1432-1971
    Keywords: Key words: Native coarctation — Balloon angioplasty — Aortic dissection
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. A 12-year-old girl underwent successful balloon angioplasty for a waist-like native coarctation. The balloon size/coarctation diameter ratio was 3.3. Postdilatation angiography showed a small aneurysm at the coarctation site. On frequent review the patient remained symptom-free and normotensive. Recatheterization was performed 14 months after balloon aortoplasty, when angiography revealed a massive aortic dissection extending from the origin of the left subclavian artery to both iliac arteries. She underwent partial replacement of the thoracic aorta. Balloon angioplasty of a narrow waist-like native coarctation may lead to extensive wall dissection and should be considered critically.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric cardiology 17 (1996), S. 118-121 
    ISSN: 1432-1971
    Keywords: Key words: Long QT syndrome — Neonatal presentation — Syncope — Ventricular arrhythmias
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. A neonate who had presented with sustained irregular heart rate during labor was found to have QT prolongation and repetitive polymorphic ventricular tachycardia (torsades de pointes) postnatally. Propranolol and propafenone successfully controlled the ventricular arrhythmias. Follow-up electrocardiograms and Holter records show persistent QT prolongation, bizarre T waves, and intermittent episodes of T wave alternans. On propranolol monotherapy the boy is thriving and completely free of ventricular arrhythmias. In the rare case of long QT syndrome in the neonate, early detection and therapy are mandatory to prevent ventricular arrhythmias and sudden death.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1971
    Keywords: Key words: Angiography — Extralobar pulmonary sequestration — Vascular connections
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. We present a case of extralobar pulmonary sequestration between the left lower lobe and diaphragm with an unusual arterial blood supply and venous drainage. Angiography revealed a large systemic artery arising from the left subclavian artery. The venous return paralleled this anomalous artery and drained into the left subclavian vein. This case illustrates the wide anatomic variability of such complex bronchovascular anomalies. Careful preoperative evaluation of both the arterial supply and venous drainage is important to avoid intraoperative complications. Angiography provides clear definition of these abnormal vascular structures, which is essential for appropriate therapeutic management.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 124 (1977), S. 217-222 
    ISSN: 1432-1076
    Keywords: Exchange transfusion ; Digoxin elimination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird von vier Fällen berichtet, bei denen sich zugleich die Indikation zur Digitalisierung und zur Austauschtransfusion ergab. Nach vorhergehender, intravenöser Digoxingabe wurde einerseits das Verhalten der Digoxinkonzentration im Plasma gemessen, andererseits die gesamte durch den Blutaustausch eliminierte Glykosidmenge bestimmt. Besondere Beachtung galt einer Abhängigkeit des eliminierbaren Prozentsatzes von dem zeitlichen Abstand der Austauschtransfusion zur vorangehenden Injektion. In allen vier Fällen wurde ein mäßiges Absinken der Plasmakonzentration beobachtet. Die durch Austauschtransfusion eliminierte Digoxinmenge stand in einem verkehrt proportionalen Verhältnis zum Intervall zwischen Digoxingabe und Blutaustausch. In keinem Fall überstieg diese eliminierte Fraktion 5% der insgesamt im Körper enthaltenen Menge.
    Notes: Abstract The report covers four cases presenting simultaneous indications for digitalisation and exchange transfusions. Intravenous administration of digoxin was followed: 1. by monitoring of the behaviour of the plasma digoxin level; 2. by determination of the total amount of glycoside eliminated by the blood exchange. Particular attention was paid to the effect of the delay between injection and exchange transfusion on the amount of digoxin eliminated. All four cases showed moderate falls in plasma levels. The amounts of digoxin eliminated by exchange transfusion were in reverse relationship to the delay between administration of digoxin and the blood exchange. At no time did the eliminated fraction exceed 5% of the total amount present in the body.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric radiology 16 (1986), S. 57-58 
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Disturbance of desmogenous ossification of the skull is an extremely rare complication of long-term prostaglandin treatment. This report describes a newborn with pulmonary atresia, ventricular septal defect and ductus-dependent pulmonary blood flow in whom administration of prostaglandin E1 (PGE1) over a period of 96 days produced uncommon clinical and radiologic findings.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1435-1803
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Das Syndrom der Pulmonalklappenaplasie mit Ventrikelseptumdefekt beinhaltet regelmäßig nicht nur eine hypo-dysplastische Pulmonalklappe und einen großen Ventrikelseptumdefekt, sondern zusätzlich auch Aneurysmen der Pulmonalarterien und ein Fehlen des Ductus arteriosus Botalli. Je nach dem Vorliegen oder Fehlen einer rechtsventrikulären Ausflußtraktobstruktion kann zwischen zwei Formen unterschieden werden. Fehlanlage der Pulmonalklappe, rechtsventrikuläre Ausflußtraktobstruktion sowie ein typischer Ventrikelseptumdefekt repräsentieren den primären Fehlbildungskomplex, welcher durch eine Septierungsstörung des Conotruncus verursacht wird. Daraus resultierendes intrauterines Herzversagen wird wahrscheinlich durch einen pränatalen Verschluß des Ductus arteriosus Botalli verhindert. Dilatation des rechtsventrikulären Ausflußtraktes und Entstehung von Pulmonalarterienaneurysmen sowie einige andere, häufig auftretende Deformitäten können dann durch diesen intrauterinen Ductusverschluß erklärt werden. Aus einer Analyse der dieses Syndrom aufbauenden, nur scheinbar unzusammenhängenden Einzeldeformitäten wird eine embryonische Entstehungstheorie abgeleitet.
    Notes: Summary 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.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1435-2451
    Keywords: Ligation of patent ductus ; Banding ; Repair of coarctation ; Shunt operation ; Infancy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Von 1970–1978 wurden 135 Säuglinge mit angeborenen Herzgefäßmißbildungen wegen Herzinsuffizienz, progredienter pulmonaler Hypertonie und schwerer Cyanose operiert. In 63% bestanden acyanotische Vitien, 68% aller Eingriffe erfolgten im ersten Lebenshalbjahr. Die Spätletalität betrug 5%, die Operationssterblichkeit 17% und war im ersten Trimenon deutlich höher als im zweiten Lebenshalbjahr. Beim häufigsten Herzfehler dem isolierten Ductus war das Operationsrisiko mit 2,5% nur geringfügig höher als bei Eingriffen im späteren Leben. Die Gesamtletalität der „Banding” Operation beim isolierten Ventrikelseptumdefekt betrug einschließlich zweizeitiger Korrektureingriffe 12,5%, bei acyanotischen Vitien insgesamt 17%. Bei einer Gesamtletalität von 26% fand sich bei Aortenisthmusstenosen eine Rezidivrate von 20%, weshalb eine Ausweitung der Indikation auf asymptomatische Stenosen abgelehnt wird. Ein höheres Operationsrisiko kennzeichnete komplexe cyanotische Anomalien sowohl hinsichtlich der „Banding” Operation (38%) als auch bei den Shuntoperationen (44%). Die möglichst frühzeitige chirurgische Intervention wird bei entsprechender Indikation empfohlen.
    Notes: Summary Between 1970 and 1978 135 infants with congenital malformations underwent operations due to congestive heart failure, progressive pulmonary hypertension, and severe cyanosis. Of them, 63% were acyanotic; 68% of all operations became necessary during the first 6 months of life. The late mortality rate was 5 % and the hospital mortality rate 17 %. A remarkably higher operative risk was found during the first 6 months of life. Ligation of patent ductus arteriosus as the most common lesion was done with a mortality rate of 2.5 % which is comparable to the risk of late repair in childhood. Pulmonary artery banding could be performed in all acyanotic malformations with a mortality rate of 17 % and of 12.5 % in isolated VSD (including debanding and VSD closure in several cases). Coarctation of the aorta was corrected with a overall mortality rate of 26%. Because of a recurrence rate of 20%, the authors think that operations are only indicated in symptomatic cases of coarctation. In complex cyanotic malformations, a higher operative risk was observed in both pulmonary artery banding (38%) and shunt operations (44%). If surgical treatment is necessary, the earlier, the better.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1435-1285
    Keywords: Key words Subaortic stenosis — accessory mitral valve tissue — echocardiography ; Schlüsselwörter Subaortenstenose — akzessorisches Mitralklappengewebe — Echokardiographie
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary An unusual case of subaortic stenosis and aortic regurgitation caused by accessory mitral valve tissue in a 10 year old boy is reported. Two-dimensional and Doppler echocardiography revealed the characteristic feature of a mobile, parachute-like mass in the left ventricular outflow tract prolapsing into the aortic valve during systole and, thus, producing a systolic pressure gradient of 70 mm Hg between the left ventricle and aorta and causing mild aortic regurgitation. The accessory valve tissue was completely excised via an aortotomy without injury to the normal mitral and aortic valves. Two-dimensional echocardiography provides excellent morphological information about the relationship between the accessory mitral valve tissue and the mitral and aortic valves, respectively. Accurate preoperative evaluation by two-dimensional echocardiography facilitates the successful surgical management of this rare condition.
    Notes: Zusammenfassung Als sehr seltene Ursache einer subvalvulären Aorteninsuffizienz wurde bei einem zehnjährigen Patienten akzessorisches Mitralklappengewebe nachgewiesen. In der zweidimensionalen Echokardiographie zeigte sich im linksventrikulären Ausflußtrakt der charakteristische Befund eines flottierenden, fallschirmartigen Gebildes, das in der Systole bis in die Aortenklappenbene hochgeschalgen wurde und einen dopplersonographischen Gradienten von 70 mm Hg zwischen linkem Ventrikel und Aorta sowie eine geringe Aorteninsuffizienz verursachte. Über eine Aortotomie konnte das akzessorische Klappengewebe vollständig ohne Schädigung der normalen Aorten- und Mitralklappe entfernt werden. Mit Hilfe der zweidimensionalen Echokardiographie ist eine detaillierte Evaluierung der morphologischen Beziehung zwischen dem akzessorischen Klappengewebe und der Mitral- bzw. Aortenklappe möglich, wodurch ein erfolgreiches, chirurgisches Vorgehen bei dieser seltenen Fehlbildung wesentlich erleichtert wird.
    Type of Medium: Electronic Resource
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