Abstract:Fabry disease is an X chromosome recessive genetic disorder. Due to the deficiency of α -galactosidase A activity, the accumulation of α-D galactosyl residue glycosphingolipids in the body eventually affects the heart, brain and kidney, resulting in multi-system diseases. Echocardiography is an effective noninvasive method to evaluate the impact of Fabry disease on cardiac structure and function, which is helpful for early diagnosis and treatment of patients. This article reviews the application value of echocardiography in assessing left ventricular structural and functional damage in Fabry patients.