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To identify predictors of survival following aortic valve replacement (AVR) in patients with low-flow and high-gradient aortic stenosis (AS).Eighty-six patients (aged 71 ± 10 years) with severe AS [aortic valve mean pressure gradient >40 mmHg or valve area <1.0 cm2] and left ventricular (LV) dysfunction [ejection fraction (EF) <50%] underwent AVR. Cox proportional hazards were used to identify independent clinical and echocardiographic predictors of mortality. Operative (30-day) mortality was 10%. Peri-operative mortality was associated with lower mean LVEF, higher mitral E:A ratio, peak systolic pulmonary artery pressure (PSPAP), and serum creatinine (by 12%, 2.3, 28 mmHg, and 74 mmol/L, respectively, all P < 0.001), NYHA class III–IV (100 vs. 65%), concomitant CABG (89 vs. 55%), urgent surgery (78 vs. 35%), and longer bypass-time (by 28 min, all P < 0.05). Mortality at 4 years was 17%. Univariate predictors [hazard ratio (HR)] of 4-year mortality were: lower EF (HR 0.68 per % increase, P < 0.001), presence of restrictive LV filling (HR: 3.52, P < 0.001), raised PSPAP (HR: 1.07, P < 0.001), and CABG (HR: 4.93, P=0.037). However, only low EF (<40%, HR 0.74, P=0.030), the presence of restrictive filling (HR 1.77, P=0.033), and raised PSPAP (>45 mmHg, HR 2.71, P=0.010) remained as independent predictors after multivariate analysis.The severity of pre-operative systolic and diastolic LV dysfunction is the major predictor of mortality following AVR for low-flow and high-gradient AS.