Geva, TalTalGevaMulder, BBMulderGavreau, KKGavreauBabu-Narayan, SVSVBabu-NarayanWald, MDMDWaldGatzoulis, MichaelMichaelGatzoulisValente, AMAMValenteHickey, KelseyKelseyHickeyPowell, Andrew JAndrew JPowellGatzoulis, Michael AMichael AGatzoulis2018-08-102018-10-19Circulation, 2018, 138 (19), pp.2106-21150009-7322http://hdl.handle.net/10044/1/61604Background -Risk factors for adverse clinical outcomes have been identified in patients with repaired tetralogy of Fallot (rTOF) before pulmonary valve replacement (PVR). However, pre-PVR predictors for post-PVR sustained ventricular tachycardia (VT) and death have not been identified. Methods -Patients with rTOF enrolled in the INDICATOR cohort-a 4-center international cohort study- who had a comprehensive preoperative evaluation and subsequently underwent PVR were included. Pre-procedural clinical, electrocardiogram, cardiovascular magnetic resonance (CMR), and postoperative outcome data were analyzed. Cox proportional hazards multivariable regression analysis was used to evaluate factors associated with time from pre-PVR CMR until the primary outcome-death, aborted sudden cardiac death, or sustained VT. Results -Of the 452 eligible patients (median age at PVR 25.8 years), 36 (8%) reached the primary outcome (27 deaths, 2 resuscitated death, and 7 sustained VT) at a median time after PVR of 6.5 years. Cox proportional hazards regression identified pre-PVR right ventricular (RV) ejection fraction < 40% (hazard ratio [HR] 2.39; 95% confidence interval [CI] 1.18 to 4.85; P = 0.02), RV mass-to-volume ratio ≥ 0.45 g/mL (HR 4.08; 95%, CI 1.57 to 10.6; P = 0.004), and age at PVR ≥ 28 years (HR 3.10; 95% CI 1.42 to 6.78; P = 0.005) as outcome predictors. In a subgroup analysis of 230 patients with Doppler data, predicted RV systolic pressure ≥40 mm Hg was associated with the primary outcome (HR 3.42; 95% CI 1.09 to 10.7; P = 0.04). Preoperative predictors of a composite secondary outcome-postoperative arrhythmias and heart failure-included older age at PVR, pre-PVR atrial tachyarrhythmias, and a higher left ventricular end-systolic volume index. Conclusions -In this observational investigation of patients with rTOF, an older age at PVR and pre-PVR RV hypertrophy and dysfunction were predictive of shorter time to postoperative death and sustained VT. These findings may inform the timing of PVR if confirmed by prospective clinical trials.© 2018 American Heart Association, Inc.Science & TechnologyLife Sciences & BiomedicineCardiac & Cardiovascular SystemsPeripheral Vascular DiseaseCardiovascular System & Cardiologyhypertrophyright ventricularpulmonary valverisk factorstachycardiaventriculartetralogy of Fallottreatment outcomeALL-CAUSE MORTALITYLONG-TERM SURVIVALMAGNETIC-RESONANCERISK-FACTORSADULTSARRHYTHMIAOUTCOMESREGURGITATIONASSOCIATIONMULTICENTERhypertrophy, right ventricularpulmonary valverisk factorstachycardia, ventriculartetralogy of Fallottreatment outcomeCardiovascular System & Hematology1103 Clinical Sciences1102 Cardiorespiratory Medicine and Haematology1117 Public Health and Health ServicesJournal Articlehttps://www.dx.doi.org/10.1161/CIRCULATIONAHA.118.034740https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.118.034740FS/11/38/28864