Management of the prolonged QT syndrome and recurrent ventricular fibrillation with an implantable automatic cardioverter‐defibrillator

Abstract

Summary:Two patients with the prolonged QT syndrome and recurrent ventricular tachyarrhythmias are presented, one of them refractory to combination antiarrhythmic drug therapy and bilateral stellate ganglion blockade. We implanted and tested in vivo an automatic cardioverterdefibrillator to provide a cardiac monitoring system with the capability of delivering a 25 J electrical discharge to the heart if rapid ventricular tachycardia or ventricular fibrillation is detected. Arrhythmia induction in the electmphysiology laboratory confirmed the appropriate recognition of the arrhythmias in each patient, with prompt discharge of the device and resultant termination of the tachycardias. We suggest that implantation of such a device may provide an effective adjunct to antiarrhythmic drug therapy in the management of infrequent, but potentially lethal, ventricular arrhythmias occurring in patients with the prolonged QT syndrome.

Genetics and Genomics of Arrhythmias

“…The ICD has been used successfully in this setting ( Groh et al, 1996 ;Platia et al, 1985 ). In some rare cases, torsades de pointes persists despite therapy with the classical modalities.…”

Atypische Form eines Long-QT-Syndroms - ein Fallbericht*

“…Ähnliches gilt für die automatischen implantierbaren Defibrillationssysteme, die wegen ihrer Größe im Kindesalter bisher nur in Einzelfällen eingesetzt werden (10), in Zukunft aber bei weiterer Ausreifung der Technologie und Verkleinerung der Aggregate eine praktikable therapeutische Alternative darstellen könnten (6).…”