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Updated: Aug 18, 2026
Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Arrhythmias in patients with congenital heart disease
1Electrophysiology Division, Department of Cardiology, Children's Hospital, Boston, Massachusetts, USA. epwalsh@cardio.tch.harvard.edu
Insights
Congenital heart disease survivors face common arrhythmias like intraatrial reentrant tachycardia (IART) and ventricular tachycardia (VT). Radiofrequency ablation and implantable defibrillator (ICD) therapy offer improved outcomes, but complex anatomy presents challenges.
Area of Science:
- Cardiology
- Cardiac Surgery
- Electrophysiology
Background:
- Improved surgical outcomes have increased the population of adult congenital heart disease (CHD) survivors.
- Cardiac arrhythmias are frequent long-term complications in CHD patients.
- Intraatrial reentrant tachycardia (IART) is the most common and challenging arrhythmia in this group.
Purpose of the Study:
- To review current treatment strategies for cardiac arrhythmias in adult congenital heart disease survivors.
- To highlight the effectiveness and challenges of radiofrequency ablation for IART.
- To discuss the role of implantable defibrillator (ICD) therapy for ventricular tachycardia (VT) in CHD.
Main Methods:
- Review of current literature on arrhythmias in congenital heart disease.
- Discussion of radiofrequency ablation techniques and outcomes for IART.
- Evaluation of implantable defibrillator (ICD) therapy for VT in CHD patients.
Main Results:
- Radiofrequency ablation achieves >90% acute success for IART, but recurrence is an issue post-Fontan operation.
- Atrial maze surgery is an alternative for recurrent IART after Fontan.
- ICD therapy improves outcomes for VT in specific CHD populations like tetralogy of Fallot.
Conclusions:
- Radiofrequency ablation and ICD therapy are valuable tools for managing arrhythmias in CHD survivors.
- Complex CHD anatomy poses unique challenges for catheterization and device implantation.
- Further multicenter studies are needed to establish objective treatment guidelines for this population.
Abstract:
Improved surgical outcome for patients with congenital heart disease (CHD) has created a rapidly expanding population of adolescents and young adult survivors. Cardiac arrhythmias are a common late sequelae of this form of heart disease. Effective treatment requires clear understanding of the underlying anatomic defect as well as the specific surgical interventions. Intraatrial reentrant tachycardia (IART) is the most common and difficult arrhythmia encountered in these patients. Traditional IART treatment with medication has been largely unsuccessful, but radiofrequency ablation has emerged in recent years as a promising option for many patients. The availability of three-dimensional mapping systems and irrigated-tip ablation catheters has improved acute success rates for IART to better than 90%. Postablation recurrence of IART still remains problematic for patients who have undergone the Fontan operation, in which case atrial maze surgery may be considered. Ventricular tachycardia (VT) is seen in a smaller number of CHD patients, most notably those with tetralogy of Fallot or aortic stenosis. The adoption of implantable defibrillator (ICD) therapy for these patients has improved outcome. Owing to their complex anatomy, the CHD population presents unique challenges during both catheterization and device implant. Multicenter study of this unique patient group is needed in order to develop more objective treatment guidelines.
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