ABC | Volume 113, Nº5, November 2019

Review Article Fernandes et al. Deposit diseases and ventricular hypertrophy Arq Bras Cardiol. 2019; 113(5):979-987 Figure 3 – Image obtained from transthoracic echocardiography. Longitudinal parasternal section showing the increased myocardial thickness of the anterior and inferior lateral septal walls of a patient with AL form amyloidosis. Bright aspect of the myocardial walls suggestive of infiltrative disease is observed. Figure 4 – Image obtained from transthoracic echocardiography. 4-chamber apical section showing a diffuse increase in the thickness of the left ventricular myocardial walls. Below, on the right, there is a parametric image of the longitudinal myocardial deformation evaluation by the speckle tracking technique. The specific treatment for FD is through ERT which, if started as early as possible, as soon as cardiac manifestations are detected and although there is no evidence yet establishing an effect on cardiovascular outcomes, may prevent the disease from developing in young people, and at least slow the progression of multiple organ dysfunction in older patients. 65-69 (Table 1) Glycogen depot disease Glycogen deposit diseases are inherited metabolic diseases of glycogen metabolism that can affect its synthesis or degradation in muscle, liver and heart tissues. 70 Danon's disease has an autosomal dominant X-linked character due to LAMP2 enzyme deficiency and the triad of heart failure with hypertrophic cardiomyopathy, skeletal myopathy and mental deficit in male patients and only cardiomyopathy in women. 71 The phenotype of cardiomyopathy is usually hypertrophic but dilated has also been described. Myopathy is usually mild with proximal weakness of the limb and cervical muscles, and nerve conduction studies show sensory and motor polyneuropathy. In male patients, the mental deficit may be observed in half of the cases and 10% in females with mild symptoms. 983