Case
A 39-day-old female infant with Ebstein's anomaly, pulmonary atresia, atrial septal defect, and patent ductus arteriosus developed repetitive critical ventricular tachycardia (VT) ranging from 200 to 240 bpm. We assumed the focal VT originated from the right ventricle (RV) from the electrocardiographic features. Pharmacological therapies and overdrive pacing were ineffective. Her cardiovascular status gradually deteriorated, so radiofrequency catheter ablation (RFCA) was performed at 46 days old, weighed only 2.9 kg, under general anaesthesia and mechanical ventilation. We approached the RV via the right internal jugular vein with 5-Fr sheath puncture. The earliest activation site was identified using bipolar electrograms and RFCA up to 60°C and 20 W for 60 s at the outflow tract of the RV terminated the arrhythmia. Ventricular tachycardia recurred at 62 days old and was terminated by RFCA. Three weeks later, Starnes' procedure, RV exclusion, division of the ductus arteriosus, construction of the Blalock-Taussig shunt, and intraoperative cryoablation for residual muscle of the excluded RV were performed. She was discharged without anti-arrhythmic agents.
Radiofrequency catheter ablation in small infants with congenital heart defects is challenging. Hence, pharmacological treatment is preferable to catheter ablation. In this case, however, RFCA was attempted as the final option because the pharmacological and pacing therapies could not manage the cardiovascular status.
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