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................................................................................ 12.2 Catheter and surgical ablation Owing to underlying anatomy and previous operations, interventional access for ablation procedures may be challenging in patients with ACHD. In addition, the nature of SVT is often atypical, and related to multiple re-entrant circuits and fibrotic atrial tissue. As a consequence, special expertise and experience with ablation of complex tachyarrhythmias and scar-related procedures are necessary.506 It is recommended that patients with complex incisional tachycardias are referred to specialist centres with adequate experience, volumes of ablation procedures, and advanced mapping capabilities. Catheter ablation procedures in the setting of ACHD are associated with lower success rates compared with the general cohort of patients with AF or atrial flutter.484

However, ablation of CTI-related arrhythmias has been reported to have a high acute success rate (>95%), although the mid-term recurrence rate may approach 20%.507 Pre-operative catheter ablation or concomitant arrhythmia surgery should be considered in patients with ACHD undergoing cardiac surgery, as its incorporation can result in improved functional class and potentially decreased requirements for chronic antiarrhythmic medication in this vulnerable population.502504

12.3 Specific disease states

12.3.1 Atrial septal defect The incidence of atrial arrhythmias in patients with atrial septal defects ranges between 515%.508 The exact impact of atrial septal defect closure, especially of late closure, on the risk of developing AT is controversial. Patients commonly present with RA MRAT. The leading mechanism is CTI-dependent tachycardia and this is generally susceptible to catheter ablation. However, CTI-dependent and ‘incisional’ atrial flutter may coexist. Closure of an existing atrial septal defect, in isolation, is generally insufficient to abolish an existing AT and catheter ablation should be considered before defect closure. Mid-term recurrence rates of 4044% have been reported in atrial septal defect patients post-flutter or AF ablation508,509; however, this should not preclude ablation procedures wherever possible.

12.3.2 Ebstein’s anomaly ATs are common in Ebstein’s anomaly, occurring in 2565% of patients.501,510512 The types of arrhythmia include atrial flutter, focal AT, and AF. In addition, 1045% of patients have right-sided APs, including WPW syndrome. More than one AP is common in this setting, and can in itself increase the risk of haemodynamic compromise and sudden cardiac death. Catheter ablation of APs has a high success rate; however, ablation procedures may be challenging and repeat procedures may be necessary as some patients have multiple ablation targets. In addition, patients may develop different arrhythmia mechanisms following ablation.501 In patients undergoing surgical repair, routine pre-operative EPS can be recommended as the diagnostic and therapeutic yield in this population is high.513

12.3.3 Transposition of the great arteries (dextrotransposition of the great arteries) after atrial switch operation (Mustard or Senning) Owing to previous surgery and scarring, atrial re-entrant tachycardias are common in patients with a Mustard or Senning repair. In addition,

Recommendations for the therapy of supraventricular tachycardia in congenital heart disease in adults

Recommendation Classa Levelb Anticoagulation for focal AT or atrial flutter should be similar to that for patients with AF.241,242,499 I C Acute therapy Haemodynamically unstable patients Synchronized DC cardioversion is recommended for haemodynamically unstable patients.8688,491,492 I B Haemodynamically stable patients Vagal manoeuvres, preferably in the supine position with leg elevation, are recommended.41,8991 I B Adenosine (618 mg i.v. bolus) is recommended if vagal manoeuvres fail.9294 I B i.v. verapamil or diltiazem should be considered, if vagal manoeuvres and adenosine fail.92,9498 IIa B i.v. beta-blockers esmolol or metoprolol) should be considered if vagal manoeuvres and adenosine fail.97,99,100 IIa C Synchronized DC cardioversion is recommended when drug therapy fails to convert or control the tachycardia.87,88 I B Chronic therapy Catheter ablation in experienced centres should be considered.292,500,501 IIa C Beta-blockers should be considered for recurrent focal AT or atrial flutter, if ablation is not possible or successful.237 IIa C In patients with SVT planned for surgical repair of a congenital heart disease anomaly, pre-operative catheter ablation or intraoperative surgical ablation should be considered.502504 IIa C Amiodarone may be considered for prevention if ablation is not possible or successful.505 IIb C Sotalol is not recommended as a first-line antiarrhythmic drug as it is related to an increased risk of pro-arrhythmias and mortality.496 III C Flecainide and propafenone are not recommended as first-line antiarrhythmic drugs in patients with ventricular dysfunction and severe fibrosis.497 III C

i.v. verapamil and diltiazem are contraindicated in the presence of hypotension or HFrEF. i.v. beta-blockers are contraindicated in the presence of decompensated heart failure. AF = atrial fibrillation; AT = atrial tachycardia; DC = direct-current; HF = heart failure; HFrEF = heart failure with reduced ejection fraction; i.v. = intravenous; SVT = supraventricular tachycardia. aClass of recommendation. bLevel of evidence Downloaded from academic.oup.com/eurheartj/article/41/5/655/5556821 by guest on 07 October 2026