A case report of a combined implantation technique of a cardioverter-defibrillator in an infant with long QT syndrome type 8 (Timothy's syndrome)

Abstract

We present a case of successful implantation of a cardioverter-defibrillator (ICD) using combined technique in a child with Timothy's syndrome. Due to high risk of sudden cardiac death (SCD) such patients often need ICD for primary or secondary prevention but implantation technique in young children remains controversial. The subcutaneous cardioverter-defibrillators could be an option in some cases, however, reliable cardiac pacing should be implemented for patients with bradyarrhythmias. An ICD implantation technique with the epicardial pacing lead placement and subcutaneous tunnel formation for endocardial defibrillation lead seems to be promising in SCD prevention also providing the opportunity for permanent pacing.

Keywords: Epicardial pacing; Subcutaneous cardioverter-defibrillator; Sudden cardiac death.

Fig. 1

Atrioventricular (AV) block 2:1 and significant QT interval prolongation on surface ECG: QT = 620 msec, RR = 960 msec, QTc = 632 msec, alternations of the T wave.

Fig. 2

Subcutaneous tunnel formation for endocardial DF-lead (left) and DF- lead delivering on a stylet through the 11 Fr introducer (right), fixating it on the sleeve to the external intercostal muscle.

Fig. 3

Pacing lead and DF-lead connection to the ICD generator.

Fig. 4

The final position of leads and ICD-device – Anterior-Posterior (left) and Lateral (right) X-ray projections.