67
PACES
Case-control design
Outcome: SCD, sustained VT, appropriate ICD shock
286 patients
70 events
Risk factor* or
†
Points
Surgical era <1980 ≥1980
Arrhythmic symptoms 3 3
≥ Moderate LV dysfunction 3 3
≥ Moderate RV dysfunction 2 1
≥ Moderate RV pressure 1 1
≥ Moderate RV volume 1 1
Age at repair <6.5 2 0
Shunt pre-repair 2 1
Complex repair 1 2
QRS duration ≥180 ms 1 1
Sustained/non-sustained VT 1 1
Total score Risk category
<3 Low
3–6 Moderate
7–9 High
>9 Very high
ACHD indicates adult congenital heart disease; BNP, B-type natriuretic peptide; CMR,
cardiovascular magnetic resonance; EF, ejection fraction; ICD, implantable cardioverter-defibrillator;
LGE, late gadolinium enhancement; LV, le ventricular; NYHA, New York Heart Association; RV,
right ventricular, RVOT, RV outflow tract; SCD, sudden cardiac death; VF, ventricular fibrillation;
and VT, ventricular tachycardia.
