[Arrhythmia in adults with congenital heart disease : Acute and long-term management].

During the last few decades, the management of cardiac arrhythmias in adult patients with congenital heart disease (CHD) has undergone a significant change from a conservative, mainly pharmacological approach, to interventional techniques such as catheter ablation or device implantation (pacemakers, implantable cardioverter defibrillators [ICD]). The long-term efficacy of these strategies is as dependent on the hemodynamic status of the patient and the type of arrhythmia as it is on the underlying heart defect and any preceding surgery. Pharmacological agents and electrical cardioversion are at the forefront of treatment for tachyarrhythmias. Individualized thromboprophylaxis plays an important role in acute and in long-term management. Catheter ablation seems to show a more favorable outcome compared with a pharmacological approach in the long-term management of supraventricular tachyarrhythmias. Surgical procedures, including arrhythmia surgery (such as the Fontan conversion operation), may improve the arrhythmia. In symptomatic patients with bradyarrhythmias, such as sinus node dysfunction, pacemaker implantation is usually indicated. In the management of ventricular arrhythmias catheter ablation is also increasingly used. This is dominated by the individual risk stratification for the prevention of sudden cardiac death (SCD). However, the ICD is still the therapeutic cornerstone in most cases. The management of cardiac arrhythmias in adult CHD patients should be performed at specialized centers where all aspects of diagnosis and treatment are covered.