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September 25, 2018 Circulation. 2018;138:e272–e391. DOI: 10.1161/CIR.0000000000000549 e346

CLINICAL STATEMENTS AND GUIDELINES TOF account for approximately 50% of ICD implantations in adult congenital heart disease.S10.8-13–S10.8-16,S10.8-40 Appropriate ICD shocks occur in up to 7.7% per year of patients with TOF receiving the ICD for primary prevention, compared with 9.8% per year in patients with a secondary prevention ICD.S10.8-20 In another study including patients with TOF as well as other lesions, inducible sustained VT did not correlate with subsequent appropriate ICD shocks.S10.8-14 Because of the high incidence of inappropriate shocks in 20% to 30% and complications in at least 30% of patients with adult congenital heart disease,S10.8-14–S10.8-17,S10.8-39,S10.8-40,S10.8-43 in addition to financial and psychological burdens, shared decision-making regarding primary prevention ICDs is essential. 7.	 In patients with recurrent sustained monomorphic VT, catheter ablation of VT can be effective.S10.8-21–S10.8-25 Hemodynamic repair, at the time that an arrhythmia is being ablated surgically, should be considered. For patients with complex adult congenital heart disease, care should be provided at experienced centers. After successful catheter ablation of VT, implantation of an ICD for those who do not have an ICD is an individualized decision based on overall functional and physiological status and shared decision making. Careful monitoring during follow-up for recurrent arrhythmias is essential. 8.	 The highest risk of SCD associated with repaired congenital heart disease reported from large contemporaneous cohorts is in patients with transposition of the great arteries with atrial baffle repair, Ebstein anomaly of the tricuspid valve, aortic stenosis, and univentricular physiology.S10.8-44–S10.8-47 Patients with Senning or Mustard atrial baffle repairs are at an increased risk for SCA, particularly during exertion.S10.8-48 The atrial baffle is noncompliant restricting ability to augment volume and may be associated with pulmonary vein stenosis and increased end-diastolic pressures. RV ischemia and infarction occur, with perfusion defects identified by myocardial perfusion studies in >40% of patients in this population.S10.8-49,S10.8-50 Risk factors for cardiac arrest in patients with transposition and atrial baffle repairs include prior ventricular septal defect closure, symptoms of HF, atrial arrhythmia, RVEF <30% to 35%, and QRS duration ≥140 ms.S10.8-48,S10.8-51 In the single multicenter study assessing outcomes after implantation of an ICD in patients with prior atrial baffle repair of transposition of the great arteries, the lack of beta blockers was associated with a high risk of appropriate ICD therapy.S10.8-26 Atrial arrhythmias frequently precede VT in transposition patients, and treatments for atrial tachycardia including catheter ablation, antitachycardia pacing algorithms, and beta blockers are important to reduce ICD shocks.S10.8-26,S10.8-52,S10.8-53

9.	 The risk of SCD is increased among patients with adult congenital heart disease compared with the general population, with the median age at death ranging from 30 to 49 years of age.S10.8-27,S10.8-44,S10.8-47,S10.8-54,S10.8-55 The risk of SCD is highest among patients with moderate or severe complexity congenital heart disease, and accounts for approximately 25% of cardiac causes of death.S10.8-5,S10.8-27,S10.8-28,S10.8-44–S10.8-46,S10.8-55,S10.8-56 Patients with septal defects and a positive family history of septal defects, cardiomyopathy, or bundle-branch block/conduction defects may have the gene mutation NKX2.5, which portends an increased risk of early SCD; genetic testing and early consideration of ICD implantation if positive is warranted.S10.8-57–S10.8-59 Patients with repaired complex forms of congenital heart disease have undergone multiple intracardiac surgeries in the first few decades of life with resultant hypertrophy and risk for subendocardial ischemia as well as scar formation contributing to VT/VF. Risk factors for SCD include increasing complexity of heart disease, VA, SVT, progressive increase in QRS duration, systemic ventricular dysfunction, and subpulmonary ventricular dysfunction.S10.8-1,S10.8-5, S10.8-6,S10.8-14,S10.8-28,S10.8-29,S10.8-36,S10.8-45–S10.8-47,S10.8-55 Extrapolation of data regarding specific measures of ventricular function warranting implantation of primary prevention ICDs from adult patients with NICM is unrealistic. The development of unexplained syncope in patients with moderate or severe complexity adult congenital heart disease may be a harbinger of risk for SCD; electrophysiological study with consideration for an ICD as primary prevention can be beneficial. 10.	 ICDs implanted in patients with adult congenital heart disease, who are in their 40s and 50s, for primary prevention indications now account for >40% to 67% of implanted devices in patients with adult congenital heart disease.S10.8-13,S10.8-15,S10.8-16,S10.8-41 In these patients, appropriate shocks are delivered in 14% to 22% of patients in the first 3 to 5 years of follow-up.S10.8-13,S10.8-15,S10.8-16 In patients with congenital heart disease and severely depressed ventricular function, or single ventricle anatomy, defibrillation threshold testing may pose excessively high risk. In patients without vascular access or prior Fontan repairs, the risk of reoperation with sternotomy for epicardial ICD implantation may outweigh the potential benefits, and consideration for transplant evaluation may be preferable. Subcutaneous implantable

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