Original Research
Predicting Survival in Repaired Tetralogy of Fallot: A Lesion-Specific and Personalized Approach

https://doi.org/10.1016/j.jcmg.2021.07.026Get rights and content
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Abstract

Objectives

This study sought to identify patients with repaired tetralogy of Fallot (rTOF) at high risk of death and malignant ventricular arrhythmia (VA).

Background

To date there is no robust risk stratification scheme to predict outcomes in adults with rTOF.

Methods

Consecutive patients were prospectively recruited for late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) to define right and left ventricular (RV, LV) fibrosis in addition to proven risk markers.

Results

The primary endpoint was all-cause mortality. Of the 550 patients (median age 32 years, 56% male), 27 died (mean follow-up 6.4 ± 5.8; total 3,512 years). Mortality was independently predicted by RVLGE extent, presence of LVLGE, RV ejection fraction ≤47%, LV ejection fraction ≤55%, B-type natriuretic peptide ≥127 ng/L, peak exercise oxygen uptake (V02) ≤17 mL/kg/min, prior sustained atrial arrhythmia, and age ≥50 years. The weighted scores for each of the preceding independent predictors differentiated a high-risk subgroup of patients with a 4.4%, annual risk of mortality (area under the curve [AUC]: 0.87; P < 0.001). The secondary endpoint (VA), a composite of life-threatening sustained ventricular tachycardia/resuscitated ventricular fibrillation/sudden cardiac death occurred in 29. Weighted scores that included several predictors of mortality and RV outflow tract akinetic length ≥55 mm and RV systolic pressure ≥47 mm Hg identified high-risk patients with a 3.7% annual risk of VA (AUC: 0.79; P < 0.001) RVLGE was heavily weighted in both risk scores caused by its strong relative prognostic value.

Conclusions

We present a score integrating multiple appropriately weighted risk factors to identify the subgroup of patients with rTOF who are at high annual risk of death who may benefit from targeted therapy.

Key Words

CMR
late gadolinium enhancement
risk stratification
sudden cardiac death
tetralogy of Fallot
ventricular tachycardia

Abbreviations and Acronyms

AUC
area under the curve
BNP
B-type natriuretic peptide
CMR
cardiovascular magnetic resonance
ECG
electrocardiogram
EF
ejection fraction
ICD
implantable cardiac defibrillator
LGE
late gadolinium enhancement
LV
left ventricle
NSVT
nonsustained ventricular tachycardia
PR
pulmonary regurgitation
rTOF
repaired tetralogy of Fallot
RV
right ventricle
RVOT
RV outflow tract
SCD
sudden cardiac death
VA
ventricular arrhythmia
VT
ventricular tachycardia

Cited by (0)

Eike Nagel, MD, served as the Guest Editor for this paper.

The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.