High-rate atrial pacing as an innovative bridging therapy in a neonate with congenital long QT syndrome
| Autor: | Ronn E. Tanel, John K. Triedman, J P Saul, Edward P. Walsh, DeLucca Jm, Fishberger Sb, Michael R. Epstein, John E. Mayer |
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| Rok vydání: | 1997 |
| Předmět: |
medicine.medical_specialty
Hyperkalemia medicine.medical_treatment Long QT syndrome QT interval Sudden death Heart Conduction System Heart Rate Physiology (medical) Internal medicine medicine Repolarization Humans Proarrhythmia business.industry Cardiac Pacing Artificial Infant Newborn Infant Arrhythmias Cardiac T wave alternans Implantable cardioverter-defibrillator medicine.disease Defibrillators Implantable Long QT Syndrome Anesthesia Cardiology Female medicine.symptom Cardiology and Cardiovascular Medicine business |
| Zdroj: | Journal of cardiovascular electrophysiology. 8(7) |
| ISSN: | 1045-3873 |
| Popis: | Neonatal Long QT Syndrome. Introduction: The neonatal presentation of the long QT syndrome is rare, although it is frequently accompanied by life-threatening arrhythmias. Infants may not survive childhood despite traditional management with beta-adrenergic blockade and pacing. Methods and Results: This case describes a newborn with a long QT interval, T wave alternans, intermittent 2:1 AV block, ventricular arrhythmias, and a family history of sudden death. After failing medical therapy, conventional VVI and DDD pacing were unsuccessful due to prolonged ventricular refractoriness and proarrhythmia. At 2 months of life, the child was treated with high-rate (180 ppm) atrial pacing to produce intentional 2:1 AV block. Following an episode of possible syncope at 16 months of age, an automatic implantable cardioverter defibrillator (ICD) was added. Finally, as recently reported, acutely induced hyperkalemia led to both a marked decrease in QTc and functional improvement in repolarization (consistent 1:1 AV conduction at rates to 180 beats/min). Spironolactone and dietary potassium were added and have produced the same effects chronically. Conclusions: High-rate atrial pacing with 2:1 AV block is presented as a novel and “bridging” therapy for neonatal long QT syndrome and 2:1 AV block with ventricular arrhythmias. Definitive therapy with ICD implantation was then possible when patient size was substantially increased. The electrophysiologic response to intentional elevation of the serum potassium suggests a genetic defect in an inward potassium channel and demonstrates a possible therapy of long QT syndrome in some future patients. |
| Databáze: | OpenAIRE |
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