Prognostic Implications of Left Ventricular Cardiomyopathy in Adults With Tetralogy of Fallot

BACKGROUND: Despite the significant risk of cardiovascular mortality after tetralogy of Fallot (TOF) repair, there are limited data about left ventricular (LV) cardiomyopathy in this population, thus creating important knowledge gaps. This study aims to address some of these knowledge gaps by describing the risk and prognostic implications of LV systolic dysfunction (LVD) after TOF repair. METHODS: We performed a cohort study of adult patients after TOF repair with an echocardiographic assessment of LV ejection fraction (LVEF) to determine the association between LVD and cardiovascular events, defined as sustained ventricular tachycardia, aborted sudden death, heart transplantation, or death. Prevalent and incidence LVD were defined as LVEF < 50% at baseline or new decrease in LVEF to < 50% during follow-up, respectively. RESULTS: Of 574 patients (age 38 ± 13 years), the baseline LVEF was 57% ± 9% and 68 (12%) had prevalent LVD. Cardiovascular events occurred in 126 patients (22%) during 10.5 ± 6.2 years of follow-up. LVEF was an independent predictor of mortality (hazard ratio, 1.16; 95% confidence interval, 1.16-1.24; P = 0.003) per 5%-point decrease in LVEF. Among the 357 patients with preserved LVEF and echocardiographic follow-up, incident LVD occurred in 23 (6%) during 3.8 ± 1.6 years of follow-up. Event-free survival was significantly lower in patients with incident LVD compared with patients without incident LVD (87% vs 71%, P = 0.021). CONCLUSION: Prevalent and incident LVD occurred in 12% and 6% of this cohort, respectively, and were associated with lower event-free survival. Incident LVD suggests the presence of subclinical LV cardiomyopathy, and further studies are required to determine optimal strategies for diagnosing and treating subclinical LV cardiomyopathy to improve outcomes in the population with TOF.