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  • Isolated congenital heart disease  (1)
  • Key words Chronic-permanent tachycardia – permanent form of junctional reciprocating tachycardia – radiofrequency catheter ablation – children – adolescents  (1)
  • 1
    ISSN: 1432-1076
    Keywords: Key words Chromosome 22q11.2 microdeletion ; Isolated congenital heart disease ; AbbreviationsAS aortic stenosis ; ASD atrial septal defect ; CHD congenital heart disease ; CoA coarctation of the aorta ; CTAFS conotruncal anomaly face syndrome ; DGS DiGeorge syndrome ; FISH fluorescence in situ hybridization ; PA pulmonary atresia ; PDA patent ductus arteriosus ; PS pulmonary stenosis ; SAS subaortic stenosis ; SPS subpulmonary stenosis ; TGA transposition of the great arteries ; TOF tetralogy of Fallot ; VCFS velo-cardio-facial syndrome ; VSD ventricular septal defect
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Microdeletions in chromosome 22q11.2 are associated with DiGeorge syndrome (DGS), velo-cardio-facial syndrome (VCFS), and several other syndromes, collectively referred to as DG/VCF. Non-dysmorphic patients with cardiac defects have also been attributed to deletions in this chromosomal region. In this study 157 consecutively catheterized patients with isolated, non-syndromic cardiac defects, and 25 patients with cardiac defects and additional stigmata (10 of whom were clinically diagnosed as DG/VCF cases prior to chromosome analysis) were analysed by fluorescence in situ hybridization with the DGS-specific probe D0832. Chromosome 22q11.2 deletions were observed only in the ten patients with the clinical diagnosis of DG/VCF. Conclusion In a large unselected cohort of patients with congenital heart disease no association between isolated or non-syndromic heart defects and the 22q11.2 microdeletion was observed. One can conclude that testing for the 22q11.2 microdeletion is clearly indicated in cases when even mild extracardiac abnormalities are present, particularly in very young infants.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1435-1285
    Keywords: Schlüsselwörter Chronisch-permanente supraventrikuläre Tachykardie – permanente Form der junktionalen Reentry-Tachykardie – atriale ektope Tachykardie – Hochfrequenzstrom-Katheterablation – Kinder und Jugendliche ; Key words Chronic-permanent tachycardia – permanent form of junctional reciprocating tachycardia – radiofrequency catheter ablation – children – adolescents
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Ten young patients with a median age of 10.5 (range: 6 to 31) years suffering from the permanent form of junctional reciprocating tachycardia (PJRT; n=7) and ectopic atrial tachycardia (AET; n=3) were treated by transcatheter radiofrequency current application. Indications for interventional therapy were failure of medical therapy after a median of 3 antiarrhythmic drugs in all patients, syncope in 1, and impaired left ventricular function in 4 patients (PJRT n=3, AET n=1). The intervention was primarily successful in all patients. Median fluoroscopy time during the ablation procedure was 17 (10 to 70) min. A median of 3 (1 to 14) radiofrequency (500 kHz) pulses was delivered at a target temperature of 70°C or with 30 W for 30 s. One patient had a recurrence of PJRT and was successfully treated by a repeat ablation. After a median follow-up of 14 (2 to 61) months, all patients are free of drug treatment and in normal sinus rhythm. Left ventricular function has normalized in patients with follow-up 〉6 months. Radiofrequency catheter ablation was a safe and effective therapy in young patients with drug-resistant forms of chronic-permanent supraventricular tachycardia.
    Notes: Zusammenfassung Zehn junge Patienten im Alter von 10,5 (Median; Streubreite 6–31) Jahren wurden wegen permanenter junktionaler Reentry-Tachykardien (PJRT, n=7) und atrialer ektoper Tachykardien (AET, n=3) katheterinterventionell mit Hochfrequenzstromablation behandelt. Indikation zur interventionellen Therapie war das Nichtansprechen auf eine medikamentöse Therapie mit Antiarrhythmika, im Median 3, bei allen Patienten. Ein Patient hatte eine Synkope erlitten, bei 4 Patienten war die linksventrikuläre Verkürzungsfraktion eingeschränkt (PJRT n=3, AET n=1). Die Katheterablation war bei allen Patienten primär erfolgreich. Der Median der Durchleuchtungszeit betrug 17 (10–17) min. Im Median wurden 3 (1–14) Hochfrequenzstromimpulse (500 kHz) bei einer Zieltemperatur von 70°C bzw. mit 30 W für 30 s appliziert. Bei einem Patienten mit PJRT wurde nach einenm Rezidiv der Tachykardie eine zweite Ablation erfolgreich durchgeführt. Während der Nachbeobachtungszeit von im Median 14 (2–61) Monaten bestand bei allen Patienten ein normaler Sinusrhythmus, bei keinem Patienten war eine medikamentöse Therapie erforderlich. Die initial eingeschränkte, linksventrikuläre Verkürzungsfraktion hatte sich bei allen Patienten mit einer Nachbeobachtungszeit 〉6 Monaten zwischenzeitlich normalisiert. Die Hochfrequenzstromablation war ein sicheres und effektives Behandlungsverfahren für junge Patienten mit medikamentös nicht beherrschbaren Formen chronisch-permanenter supraventrikulärer Tachykardie wie der junktionalen Reentry-Tachykardie und der atrialen ektopen Tachykardie.
    Type of Medium: Electronic Resource
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