Congenital complete heart block associated with QT prolongation

Area of Science:

• Cardiology
• Electrophysiology
• Pediatric Cardiology

Background:

• Congenital complete heart block and QT prolongation are rare arrhythmias.
• Their coexistence presents unique electrophysiological challenges.

Purpose of the Study:

• To investigate the electrophysiological and clinical features of congenital complete heart block with QT prolongation.
• To evaluate treatment outcomes for this specific arrhythmia syndrome.

Main Methods:

• Studied eight patients with congenital complete heart block and QT prolongation.
• Compared findings with 22 control patients with congenital complete heart block only.
• Analyzed ventricular tachycardia, QT intervals, and syncopal episodes.

Main Results:

• The study group frequently exhibited torsade de pointes ventricular tachycardia, often provoked by ventricular extrastimuli.
• Markedly prolonged corrected QT intervals were observed, shortening with pacing but remaining elevated.
• All patients in the study group experienced syncopal attacks, unlike the control group.

Conclusions:

• Congenital complete heart block with QT prolongation is associated with a high risk of polymorphic ventricular tachycardia and syncope.
• Pacemaker implantation and beta-blocker therapy led to favorable outcomes, preventing further events.