Arrhythmia in Congenital Heart Disease
Arrhythmia in Congenital Heart Disease
Published: June 2007
Before the advent of surgery only 20% of children with congenital heart disease survived to adult life.1 Great achievements in paediatric cardiology and cardiac surgery over the last few decades resulted in an increased survival of children with congenital heart disease (CHD). Today we are facing the first generation of grown-up congenital heart disease (GUCH) patients and the management of their clinical problems represents a new complicated task for paediatricians, cardiologists, cardiac surgeons and electrophysiologists. This field being so new, there are few data about the epidemiology of this population; from the available information we know that it is growing quickly and that there is a significant increase in the number of patients with complex lesions.2Arrhythmia is the main cause of morbidity and mortality in GUCH patients, leading to a high rate of hospitalisation in this population. Arrhythmogenic substrate may exist congenitally in association with structural anomalies or it can be acquired secondary to natural history (cardiac defects, haemodynamic changes, degenerative fibrosis) or to surgical repair (scarring, postoperative haemodynamic abnormalities).3
Little is known about the effectiveness of antiarrhythmic drug therapy in this population and we therefore have to apply the information obtained by trials carried out on general populations or on patients with noncongenital cardiomyopathies. Any clinician dealing with arrhythmias in GUCH patients recognises the low success rate of antiarrhythmic drugs (AADs); furthermore, it is difficult to plan lifelong drug therapy in young patients, especially with concern about the possible effects of most AADs on myocardial contractility.
It must be emphasised that rhythm disturbances that are not harmful in the general population might be critical in GUCH patients. Supraventricular arrhythmia is the most frequent problem, and it might cause invalidating and refractory heart failure in otherwise well patients. There is a lower incidence of ventricular arrhythmia, but it might be life-threatening at first presentation; for this reason, the medical community is making a great effort to identify an effective risk stratification strategy for these patients.
Complete correction of residual haemodynamic abnormalities is the firstline treatment for arrhythmia. Patients presenting with an arrhythmic problem despite the best possible haemodynamic correction can be treated by catheter ablation, intra-operative ablation or device implantation.
Transcatheter Ablation
In the past, catheter ablation was limited by technical problems. Poor understanding of peculiar anatomy in these patients makes for arduous catheter manipulation. Arrhythmogenic substrate is often complicated, presenting with multiple circuits maintaining arrhythmias. In the presence of increased wall thickness it is difficult to obtain a transmural lesion by catheter ablation. Today, technical implementation and better physiopathological knowledge allow the transcatheter (TC) approach to be effective in treating arrhythmias in GUCH patients.
Using a sophisticated electroanatomical mapping system, we are able to characterise the electrophysiological properties of the chamber of interest, identifying sites of conduction block or slowing. This information, together with the understanding of arrhythmia mechanisms, allows identification of optimal ablation sites.4,5
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- 5 August 2010
- 28 August 2010






