Risk Scores for Calculating Risk for Sustained Ventricular Tachycardia/Sudden Cardiac Death in Repaired Tetralogy of Fallot

PREVENTION-ACHD

Coronary artery disease

NYHA class II-III symptoms

Supraventricular tachycardia

Systemic EF < 40%

Subpulmonary EF < 40%

QRS duraton ≥ 120 ms

QT disperson ≥ 70 ms

Brompton, UK

RV LGE extent

Minimal/mild extent (0)

Moderate (+22)

Severe (+24)

LV LGE present

Yes (+11)

No (0)

RVEF

> 47% (0)

36% - 47% (+8)

≤ 35% (+16)

LVEF

> 55% (0)

36% - 55% (+5)

≤ 35% (+17)

Others

Peak VO2 ≤ 17 mL/kg/m² (+9)

BNP ≥ 127 ng/L (+9)

Akinetic RVOT length ≥ 55 mm (+9)

RV pressure ≥ 47 mmHg (+5)

PACES

Surgical era ≥ 1980

Surgical Era < 1980

Surgical Era ≥ 1980

Result

Total score: 0 score
Annual SCD rate: <1 %

References

1) Vehmeijer JT, Koyak Z, Leerink JM, Zwinderman AH, Harris L, Peinado R, et al. Identification of patients at risk of sudden cardiac death in congenital heart disease: The PRospEctiVE study on implaNTable cardIOverter defibrillator therapy and suddeN cardiac death in Adults with Congenital Heart Disease (PREVENTION-ACHD). Heart Rhythm. 2021 May 1;18(5):785–92. doi:10.1016/j.hrthm.2021.01.009

2) Ghonim S, Gatzoulis MA, Ernst S, Li W, Moon JC, Smith GC, et al. Predicting Survival in Repaired Tetralogy of Fallot: A Lesion-Specific and Personalized Approach. JACC: Cardiovascular Imaging. 2022 Feb 1;15(2):257–68. doi:10.1016/j.jcmg.2021.07.026

3) Atallah J, Gonzalez Corcia MC, Walsh EP. Ventricular Arrhythmia and Life-Threatening Events in Patients With Repaired Tetralogy of Fallot. The American Journal of Cardiology. 2020 Oct 1;132:126–32. doi:10.1016/j.amjcard.2020.07.012

4) Writing Committee Members∗, Gurvitz M, Krieger EV, Fuller S, Davis LL, Kittleson MM, et al. 2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2026 Feb 24;153(8):e115–251. doi:10.1161/CIR.0000000000001402

Established Risk Factors for Sustained Monomorphic Ventricular Tachycardia/Sudden Cardiac Death in Repaired Tetralogy of Fallot [4]

QRS duration ≥ 180 ms
Moderate or greater LV or RV dysfunction
Prior ventriculotomy
Late age at definitive repair
Symptomatic nonsustained ventricular tachycardia/arrhythmia symptoms
QRS fragmentation
Inducible sustained ventricular arrhythmia at invasive electrophysiology study