p.73 抽出テキスト

September 25, 2018 Circulation. 2018;138:e272–e391. DOI: 10.1161/CIR.0000000000000549 e344

CLINICAL STATEMENTS AND GUIDELINES

it may inadequately prevent the risk of subsequent VT or SCA. The use of ICDs in adult congenital heart disease patients for secondary prevention accounts for approximately 50% of implantations presently, at a mean age of 36 to 41 years.S10.8-13–S10.8-17 Patients with adult congenital heart disease experience appropriate shock rates of 3% to 6% per year, with equivalent or slightly increased frequency of appropriate shocks for secondary prevention indications.S10.8-14,S10.8-15,S10.8-17

Table 11.  Congenital Heart Disease: Risk Factors for VA/SCD

Congenital Heart Disease Incidence of VA Incidence of SCD Higher Risk Characteristics Simple complexity ASDS10.8–44,S10.8–47,S10.8–57—S10.8–62 2%–6% <1.5% Ventricular pacing RV dilatation Pulmonary hypertension NKX2.5 gene VSDS10.8–27,S10.8–44,S10.8–47,S10.8–57—S10.8–63 3%–18% <3% Moderate complexity Tetralogy of FallotS10.8-1,S10.8-2,S10.8-5,S10.8-6,S10.8–28,S10.8–34,S10.8–36,S10.8–

44,S10.8–46,S10.8–47,S10.8–54—S10.8–56,S10.8–62—S10.8–65 14%–31% 1.4%–8.3% Unexplained syncope Frequent or complex VA Sustained VT QRS duration ≥180 ms Inducible sustained VT Atrial tachycardia Decreased LVEF Dilated right ventricle Severe PR Severe PS Aortic stenosis S10.8–27,S10.8–44,S10.8–56 10%–34% 3%–20% Unexplained syncope Severe LV hypertrophy Aortic stenosis mean pressure gradient >40 mm Hg Ventricular dysfunction Coarctation of aortaS10.8–28,S10.8–29,

S10.8–44,S10.8–46,S10.8–56,S10.8–62 2% 2% Aneurysm at repair site Aortic stenosis Systemic hypertension Premature coronary artery disease Ebstein’s anomalyS10.8–45,S10.8–47,S10.8–55 2% 3%–6% Cardiomegaly Atrial fibrillation Wide complex tachycardia Mitral regurgitation Dilated RVOT Severe complexity Transposition of the great arteriesS10.8–27,S10.8–44—S10.8–48,S10.8–

51,S10.8–55,S10.8–56,S10.8–62 Atrial switch

Atrial switch 2% 3%–9.5% Mustard repair Arterial switch 2% 1% Prior VSD closure cc-TGA 10% 17%–25% Unexplained syncope Atrial tachycardia Coronary orifice stenosis Systemic ventricular dysfunction Severe tricuspid regurgitation Truncus arteriosusS10.8–66,S10.8–67 10% 4% Multiple surgical repairs Coronary anomalies Ventricular dysfunction and/or hypertrophy Fontan repair for univentricular physiology*S10.8–27,S10.8–37, S10.8–44,S10.8–45,S10.8–47,S10.8–55,S10.8–68 5%–17% 2.8%–5.4% Atrial tachycardia Longer duration of follow-up Ascites Protein-losing enteropathy *Univentricular physiology includes: Tricuspid atresia, Double inlet left ventricle, Mitral atresia, Hypoplastic left heart, Unbalanced AV septal defect. ASD indicates atrial septal defect; cc-TGA, congenitally corrected transposition of the great arteries; LV, left ventricular; LVEF, left ventricular ejection fraction; PR, pulmonary regurgitation; PS, pulmonary stenosis; RV, right ventricular; RVOT, right ventricular outflow tract; SCD, sudden cardiac death; VA, ventricular arrhythmia; VSD, ventricular septal defect; and VT, ventricular tachycardia.

Downloaded from http://ahajournals.org by on October 7, 2026