Abstract Background Cardiovascular disease is the most common cause of death in patients with end-stage kidney disease on haemodialysis. The potential clinical consequence of systematic echocardiographic assessment is however not clear. In an unselected, contemporary population of patients on maintenance haemodialysis we aimed to assess: the prevalence of structural and functional heart disease, the potential therapeutic consequences of echocardiographic screening and whether left-sided heart disease is associated with prognosis. Methods Adult chronic haemodialysis patients in two large dialysis centres had transthoracic echocardiography performed prior to dialysis and were followed prospectively. Significant left-sided heart disease was defined as moderate or severe left-sided valve disease or left ventricular ejection fraction (LVEF) ≤40%. Results Among the 247 included patients (mean 66 years of age [95%CI 64–67], 68% male), 54 (22%) had significant left-sided heart disease. An LVEF ≤40% was observed in 31 patients (13%) and severe or moderate valve disease in 27 (11%) patients. The findings were not previously recognized in more than half of the patients (56%) prior to the study. Diagnosis had a potential impact on management in 31 (13%) patients including for 18 (7%) who would benefit from initiation of evidence-based heart failure therapy. After 2.8 years of follow-up, all-cause mortality among patients with and without left-sided heart disease was 52 and 32% respectively (hazard ratio [HR] 1.95 (95%CI 1.25–3.06). A multivariable adjusted Cox proportional hazard analysis showed that left-sided heart disease was an independent predictor of mortality with a HR of 1.60 (95%CI 1.01–2.55) along with age (HR per year 1.05 [95%CI 1.03–1.07]). Conclusion Left ventricular systolic dysfunction and moderate to severe valve disease are common and often unrecognized in patients with end-stage kidney failure on haemodialysis and are associated with a higher risk of death. For more than 10% of the included patients, systematic echocardiographic assessment had a potential clinical consequence.