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abstractpubmed· Abstract 2019· item PMID:31109889

Echocardiography in Infiltrative Cardiomyopathy. Left ventricular (LV) wall thickening can occur due to both physiological and pathological processes. Some LV wall thickening is caused by infiltrative cardiac deposition diseases - rare disorders from both inherited and acquired conditions, with varying systemic manifestations. They portend a poor prognosis and are generally not reversible except in rare circumstances when early diagnosis and treatment may alter the outcome (e.g., Fabry disease). Cardiac involvement is variable and depends on the degree of infiltration and type of infiltrate. These changes often lead to the development of abnormalities in both the relaxation and contractile function of the heart ultimately resulting in heart failure. Echocardiography is generally the first investigation of choice as it is easily available and gives valuable information about the thickness of the ventricular walls as well as systolic and diastolic function. It is also able to identify unique, characteristic features of the disease as well as detecting any haemodynamic sequelae. This review looks at the role of echocardiography in the diagnosis and prognosis of infiltrative cardiac deposition diseases.