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Circulation. 2018;138:e272–e391. DOI: 10.1161/CIR.0000000000000549 September 25, 2018 e345 CLINICAL STATEMENTS AND GUIDELINES

Patients with adult congenital heart disease experience a higher rate of complications and inappropriate shocks compared with other adult populations.S10.8-13–S10.8-17,S10.8-39 4.	 Challenges of ICD implantation in patients with adult congenital heart disease may include anatomic complexity, intracardiac shunts, and limited vascular access to the ventricle. Patients with adult congenital heart disease receiving an ICD have an increased rate of complications of 26% to 45%, as well as inappropriate shocks in 15% to 25% of patients.S10.8-13–S10.8-16,S10.8-40 Limited studies on the use of subcutaneous implantable cardioverterdefibrillator implantation, particularly in patients with single ventricle anatomy,S10.8-41 report improved success by using right in addition to left parasternal lead positioning for screening.S10.8-42 Patients with a single ventricle or a systemic right ventricle may not tolerate defibrillation threshold testing, resulting in multiorgan system failure. Patients with complex anatomy, such as older patients with univentricular physiology, or patients with significantly reduced ventricular function, marked hypertrophy, or multiple prior surgeries, may benefit from earlier consideration of heart transplantation before renal or liver dysfunction progresses. 5.	 Patients with repaired TOF who are at an increased risk of sustained VT include those with prior palliative systemic to pulmonary shunts, unexplained syncope, frequent PVCs, atrial tachycardia, QRS duration  ≥180  ms, decreased LVEF or diastolic dysfunction, dilated right ventricle, severe pulmonary regurgitation or stenosis, or elevated levels of BNP. Patients with TOF physiology and suboptimal hemodynamic status are more likely to have inducible sustained VT,S10.8-18,S10.8-19,S10.8-33,S10.8-35 and inducible sustained VT correlated with an increased risk of SCA in a multicenter cohort study.S10.8-19 Evaluation of hemodynamics for residual abnormalities is important, with catheter or surgical treatment of important lesions prior to consideration of ICD implantation. 6.	 In a multicenter cohort, inducible sustained VT in patients with TOF was an independent risk factor for subsequent clinical VT or SCDS10.8-19; patients in that early study had cardiomegaly and prior palliative shunts. Patients with repaired

Figure 16. Prevention of SCD in patients with adult congenital heart disease. Colors correspond to Class of Recommendation in Table 1. See Section 10.8 for discussion. *High-risk features: prior palliative systemic to pulmonary shunts, unexplained syncope, frequent PVC, atrial tachycardia, QRS duration ≥180 ms, decreased LVEF or diastolic dysfunction, dilated right ventricle, severe pulmonary regurgitation or stenosis, or elevated levels of BNP. †Frequent VA refers to frequent PVCs and/or nonsustained VT. ACHD indicates adult congenital heart disease; BNP, B-type natriuretic peptide; EP, electrophysiological; ICD, implantable cardioverter-defibrillator; LVEF, left ventricular ejection fraction; PVC, premature ventricular complexes; SCD, sudden cardiac death; TOF, tetralogy of Fallot; VA, ventricular arrhythmia; and VT, ventricular tachycardia.

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