Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, with a familial
recurrence risk of 3%. Despite performing an optimal surgical repair, TOF patients may feature
a poor medium and long-term survival rate: atrial re-entrant tachycardia will develop in more than 30%
of patients and high-grade ventricular arrhythmias will be seen in about 10% of patients.
These life-threatening arrhythmias and consequent sudden death continue to represent serious complications
following TOF repair. Radiofrequency ablation and implantable cardioverter defibrillator are today
the most effective therapeutic tools in these subjects, while the administration of antiarrhythmic drugs
(Ib agents, beta blockers, and amiodarone), widely prescribed in the past, is now limited to few conditions.
However pharmacological therapy still plays a role in the management of those patients who are
resistant to the above stated invasive electrophysiological treatments.
Keywords: Tetralogy of Fallot, congenital heart disease, sudden death, follow up, ventricular arrhythmias, antiarrhythmic
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