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A defibrillator using high voltage pericardial patches was implanted in a 30 month old girl with the long QT syndrome and out of hospital cardiac arrest despite β blockers (left). During the first three years she remained well on atenolol 25 mg twice daily with no shocks or syncope. She then had five successive shocks within two weeks because of inappropriate T wave sensing. A chest x ray revealed that owing to growth the pericardial patches had been displaced upwards and were positioned across the atria (right). It could not be certain that the patches were in a satisfactory position to defibrillate the ventricles. In addition, the ventricular lead had lost its original atrial loop and was stretched across the tricuspid valve. The defibrillator was therefore revised to a transvenous system using a single 9 F pacing/sensing/defibrillator lead, and the lead tunnelled to an active generator implanted in the abdomen. The pericardial patches were left in situ to avoid thoracotomy. Defibrillation thresholds between the abdominal generator and ventricular lead were 10−15 Joules.
Our patient illustrates that growth affects external cardiac patches as well as transvenous leads, and the positions of both should be monitored at regular intervals by chest radiography. With the development of smaller leads and active generators, transvenous implantable cardioverter defibrillator systems can be used even for small children.