JACC. Cardiovascular imaging | 2021
Predicting Survival in Repaired Tetralogy of Fallot: A Lesion-Specific and Personalized Approach.
Abstract
OBJECTIVES\nWe sought to identify patients with repaired tetralogy of Fallot (rTOF) at high risk of death and malignant ventricular arrhythmia (VA).\n\n\nBACKGROUND\nTo date there is no robust risk stratification scheme to predict outcomes in adults with rTOF.\n\n\nMETHODS\nConsecutive 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.\n\n\nRESULTS\nThe 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\xa0≤47%, LV ejection fraction\xa0≤55%, B-type natriuretic peptide\xa0≥127\xa0ng/L, peak exercise oxygen uptake (V02)\xa0≤17\xa0mL/kg per minute, prior sustained atrial arrhythmia, and age\xa0≥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\xa0< 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\xa0≥55\xa0mm and RV systolic pressure\xa0≥47\xa0mm\xa0Hg identified high-risk patients with a 3.7% annual risk of VA (AUC: 0.79; P\xa0< 0.001) RVLGE was heavily weighted in both risk scores caused by its strong relative prognostic value.\n\n\nCONCLUSIONS\nWe 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.