ABSTRACT
Ebstein's anomaly is a rare and complex congenital heart disease involving abnormal embryological development of the tricuspid valve. Its exact prevalence remains unknown given the under-diagnosis of minor forms and the broad clinical spectrum of this pathology resulting from the highly variable tricuspid morphology. Echocardiography plays an important role in diagnosis, assessment and prognostic evaluation of this condition. We present the case of a 6-year-old boy born from a twin pregnancy with a severe neonatal EA diagnosed by ultrasound at 29 weeks of gestation and we discuss the management on the light of the recent literature.
L'anomalie d'Ebstein est une cardiopathie congénitale complexe et rare impliquant le développement embryologique anormal de la valve tricuspide (VT). Sa prévalence exacte reste inconnue étant donné le sous-diagnostic des formes mineures et le large spectre clinique de cette pathologie résultant de la haute variabilité de la morphologie tricuspidienne. L'échocardiographie occupe une place importante dans le diagnostic, la mise au point et l'évaluation pronostique de cette affection. Nous rapportons le cas d'un patient de 6 ans issu d'une grossesse gémellaire présentant une malformation d'Ebstein néonatale sévère, diagnostiquée par échographie à la 29ème semaine d'aménorrhée, et nous discutons la prise en charge à la lumière de la littérature récente.
Subject(s)
Ebstein Anomaly , Child , Ebstein Anomaly/diagnosis , Ebstein Anomaly/surgery , Echocardiography , Female , Humans , Male , Pregnancy , Prenatal Diagnosis , Tricuspid ValveABSTRACT
Adenosine is an endogenous nucleoside acting on coronary perfusion and myocardial conduction. Although the anti-arrhythmic effects of adenosine have been known for decades, interest in the use of adenosine or adenosine-triphosphate (ATP) (a precursor of adenosine) in termination of supraventricular tachycardia (SVT) has been renewed. We studied the use of striadyne (ATP and a mixture of other nucleosides including adenosine) in 22 infants younger than 6 months in order to evaluate efficiency and safety of the drug in this particular age group. Striadyne stopped SVT in 17 cases and was diagnostic in another 4 cases. Ten out of 17 successfully converted infants showed one or more reinitiations of SVT, which were easily controlled. The results support the efficiency of ATP for the termination of re-entry types of tachycardia, as well as its diagnostic value and its lack of serious side-effects.
Subject(s)
Adenosine Triphosphate/therapeutic use , Anti-Arrhythmia Agents/therapeutic use , Tachycardia, Supraventricular/drug therapy , Adenosine Triphosphate/administration & dosage , Anti-Arrhythmia Agents/administration & dosage , Child, Preschool , Electrocardiography , Humans , Infant , Infant, Newborn , Injections, Intravenous , Tachycardia, Supraventricular/diagnosis , Treatment OutcomeABSTRACT
UNLABELLED: Adenosine is an endogenous nucleoside acting on coronary perfusion and myocardial conduction. Although the anti-arrhythmic effects of adenosine have been known for decades, interest in the use of adenosine or adenosine triphosphate (ATP- a precursor of adenosine) in termination of supraventricular tachycardia (SVT) has been renewed. We studied the use of Striadyne (ATP and a mixture of other nucleosides including adenosine) in 22 infants younger than 6 months in order to evaluate efficacy and safety of the drug in this particular age group. Striadyne stopped SVT in 17 cases and was diagnostic in another 4 cases. Ten out of 17 successfully converted infants showed one or more reinitiations of SVT, which were easily controlled. The results support the efficacy of ATP for the termination of re-entry types of tachycardia, as well as its diagnostic value and its lack of serious side-effects. CONCLUSION: Adenosine or ATP could be the drug of choice for the treatment of junctional tachycardia in infants after vagal manoeuvres have failed. Provided continuous ECG monitoring is performed during injection, adenosine or ATP may be diagnostic in other types of supraventricular tachycardia and even in wide-complex tachycardia. We suggest a bolus injection of 0.5-1.0 mg/kg to be used prior to transport to the paediatric cardiology unit.
Subject(s)
Adenosine Triphosphate/therapeutic use , Anti-Arrhythmia Agents/therapeutic use , Tachycardia, Supraventricular/drug therapy , Adenosine Triphosphate/administration & dosage , Anti-Arrhythmia Agents/administration & dosage , Child, Preschool , Electrocardiography , Humans , Infant , Infant, Newborn , Injections, Intravenous , Tachycardia, Supraventricular/diagnosis , Treatment OutcomeABSTRACT
Foetal cardiology is now a recognised chapter of both paediatric cardiology and foetal medicine. We describe here our experience with antenatal diagnosis of cardiac malformations and discuss both its genetic and epidemiological issues. Our experience confirms if need be that foetal medicine requires a well organised first-level foetal evaluation.
Subject(s)
Echocardiography/methods , Heart Defects, Congenital/diagnostic imaging , Abnormalities, Multiple , Female , Humans , Predictive Value of Tests , Pregnancy , Prognosis , Reproducibility of Results , Ultrasonography, PrenatalABSTRACT
Deep abdominal palpation was performed prospectively in 900 newborns. Normal kidneys were felt in all but 8. 2 of these had a normal ultrasonography, 2 had an obstructive hydronephrosis, 1 had bilateral renal hypoplasia and 3 had a previously undiagnosed unilateral renal agenesis. Despite the increasing use of prenatal ultrasonography, careful abdominal examination should be performed in all newborns.
