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Circulation. 2018;138:e272–e391. DOI: 10.1161/CIR.0000000000000549 September 25, 2018 e343 CLINICAL STATEMENTS AND GUIDELINES I B-NR 4. In patients with adult congenital heart disease with SCA due to VT or VF in the absence of reversible causes, an ICD is recommended if meaningful survival of greater than 1 year is expected.S10.8-13–S10.8-17 IIa B-NR 5. In adults with repaired tetralogy of Fallot physiology with high-risk characteristics and frequent VA, an electrophysiological study can be useful to evaluate the risk of sustained VT/VF.S10.8-18,S10.8-19 IIa B-NR 6. In adults with repaired tetralogy of Fallot physiology and inducible VT/VF or spontaneous sustained VT, implantation of an ICD is reasonable if meaningful survival greater than 1 year is expected.S10.8-1,S10.8-19,S10.8-20 IIa B-NR 7. In patients with adult congenital heart disease with recurrent sustained monomorphic VT or recurrent ICD shocks for VT, catheter ablation can be effective.S10.8-21–S10.8-25 IIa B-NR 8. In adults with repaired severe complexity adult congenital heart disease and frequent or complex VA, a beta blocker can be beneficial to reduce the risk of SCA.S10.8-26 IIa B-NR 9. In patients with repaired moderate or severe complexity adult congenital heart disease with unexplained syncope and at least moderate ventricular dysfunction or marked hypertrophy, either ICD implantation or an electrophysiological study with ICD implantation for inducible sustained VA is reasonable if meaningful survival of greater than 1 year is expected.S10.8-5,S10.8-16,S10.8-27–S10.8-29 IIb B-NR 10. In patients with adult congenital heart disease and severe ventricular dysfunction (LVEF <35%) and symptoms of heart failure despite GDMT or additional risk factors, ICD implantation may be considered if meaningful survival of greater than 1 year is expected.S10.8-14–S10.8-16,S10.8-20 III: Harm B-NR 11. In patients with adult congenital heart disease who have asymptomatic VA, prophylactic antiarrhythmic therapy with class Ic medications (ie, flecainide, propafenone) or amiodarone is potentially harmful.S10.8-30–S10.8-32 Synopsis Tetralogy of Fallot (TOF) is defined as, congenital heart disease with RVOT obstruction and ventricular septal defect, often requiring right ventricle to pulmonary artery conduit placement or pulmonary valve replacement; includes TOF and double-outlet right ventricle. Moderate complexity congenital heart disease is defined as congenital heart disease requiring intracardiac surgical repair, other than isolated atrial and ventricular septal defects; includes TOF, aortic stenosis, coarctation of aorta, and Ebstein anomaly of the tricuspid valve. Severe complexity congenital heart disease is defined as cyanotic congenital heart disease requiring intracardiac repair in infancy, often with staging procedures; includes transposition of the great arteries, truncus arteriosus, and single ventricle anatomy (Figure 16). Recommendation-Specific Supportive Text 1. The association of VT with RV hemodynamic abnormalities was first established in patients with repaired TOF.S10.8-33 Multiple studies since that time have demonstrated the correlation of hemodynamic residue and ventricular dysfunction with risk of VT or SCD in patients with congenital heart disease.S10.8-1,S10.8-3–S10.8-6,S10.8-18,S10.8-34–S10.8-36 Presentation with frequent or complex VA may indicate worsening hemodynamic function, coronary artery compromise, or decreased perfusion in the setting of ventricular hypertrophy. Evaluation may also include exercise testing to assess functional capacity.S10.8-35 Careful evaluation of hemodynamic status for optimization of management is important.S10.8-9 Potentially treatable residual hemodynamic problems may be identified during hemodynamic evaluation, such as outflow tract stenosis or significant regurgitation, which may benefit from either catheter or surgical intervention.S10.8-3,S10.8-7,S10.8-10,S10.8-12,S10.8-37 Patients with markedly reduced ventricular function, elevated end-diastolic pressures, or pulmonary hypertension should be treated for underlying hemodynamic problems as part of their arrhythmia management. 2. The correlation of residual hemodynamic abnormalities with VA has been most extensively studied in patients with repaired TOF, where RV hypertension, residual pulmonary outflow tract obstruction or regurgitation, and RV dilation are risk factors for VT/SCD.S10.8-1,S10.8-2,S10.8-4,S10.8-8,S10.8-33,S10.8-34,S10.8-36 In these studies, frequent PVCs correlated with risk of clinical or inducible sustained VT. A combined approach of surgery for structural abnormalities with map-guided arrhythmia surgery has been used with success,S10.8-3,S10.8-8,S10.8-10,S10.8-12 but elimination of VT circuits may be limited by deep endocardial or LV origin of VT and limitations of operative mapping; an empiric approach to VT surgery is generally not recommended as it has limited effectiveness and carries risk of ventricular proarrhythmia.S10.8-38 Pulmonary valve replacement in patients with TOF may result in improved hemodynamics and functional status, but it may not eliminate the risk of VTS10.8-3,S10.8-12; postoperative reassessment for the need for an ICD is performed after the early recovery period. 3. Correction of residual hemodynamic/structural abnormalities contributing to VT may improve ventricular function and reduce symptoms, but Recommendations for Adult Congenital Heart Disease (Continued) COR LOE Recommendations Downloaded from http://ahajournals.org by on October 7, 2026