ABC | Volume 113, Nº1, July 2019

Statement Position Statement on Indications of Echocardiography in Adults – 2019 Arq Bras Cardiol. 2019; 113(1):135-181 by the 2D TTE (Simpson method). It is desirable that the evaluation by GLS and TnI is carried out. If not possible to perform GLS, report the S-wave of the medial and lateral tissue Doppler of the mitral annulus. Further monitoring of left ventricular function is recommended after this initial evaluation, depending on the chemotherapy to be initiated. b) Type I drugs (anthracyclines): evaluate left ventricular function (2D/3D LVEF and GLS) at the end of chemotherapy and after 6 months at dose < 240 mg/m². For doses > 240 mg/ m², evaluate left ventricular function before each additional 50 mg/m² cycle at the end of chemotherapy and after 6 months. c) Type II drugs (trastuzumab): evaluate left ventricular function every three months during chemotherapy. d) Patients receiving trastuzumab following anthracycline treatment: assess left ventricular function every three months during chemotherapy and six months after its completion. 2.3.6. Hypertrophic Cardiomyopathy Hypertrophic cardiomyopathy (HCM) is a genetic cardiovascular disease characterized by increased left ventricular wall thickness ≥ 15 mm in adults, with non- dilated ventricular cavity not explained by abnormal loading conditions, such as arterial hypertension or valve aortic stenosis. 72 Minor hypertrophy degrees (13 to 14 mm) may also diagnose HCM, particularly among relatives of these patients. TTE is considered the initial imaging for diagnosis, stratification of the risk of cardiac events and management of patients with HCM. Among the parameters to be evaluated in HCM are: location and hypertrophy degree; identification of obstruction and intraventricular gradient at rest or intentionally caused; presence of magnitude of mitral reflux; systolic and diastolic function; and LA size. Any hypertrophy pattern may be found, though the asymmetric is the most frequent one (75% of cases), and it is more common at the confluence of the anterior interventricular septum with the LV free wall. 73 Other forms of hypertrophy are: basal, concentric, apical and lateral wall. There is a linear association between maximum myocardial thickness and sudden death, with greater risk in patients with thickness ≥ 30 mm. 72,74 Gradual identification of the LV outflow tract is important in the management o symptoms and in the stratification of the risk of sudden death. 72 TTE evaluation generally characterizes the presence of LV outflow tract obstruction (instantaneous gradient ≥ 30 mmHg) at rest (one third of patients) of after provocative maneuvers (one third), such as exercises (echocardiography under physical stress) or Valsalva maneuver. Echocardiography under physical stress can be very useful in patients with HCM, since in addition to detecting the presence and degree of obstruction during effort, it allows the objective evaluation of symptoms, functional capacity, systolic blood pressure response and the presence of secondary mitral regurgitation. Approximately 25% of HCM patients have abnormal blood pressure response during exercise, characterized by a drop in systolic pressure or by failure to increase in > 20 mmHg. This finding has been interpreted as a risk factor for unfavorable prognosis and sudden death. 75 Stress echocardiography with dobutamine is not recommended. The cut-off value of the intraventricular gradient ≥ 50 mmHg at rest or after provocative maneuvers is considered when indicating surgical treatment or percutaneous intervention in symptomatic patients, despite therapy with optimized medication. 72 Patients with HCM in general have diastolic dysfunction, commonly with altered relaxation (grade I), though without significant correlation between mitral flow data and LV filling pressures. Thus, the integrated approach of mitral Doppler data, tissue Doppler, pulmonary vein flow and LA volume is recommended in these patients. 76 LVEF is normal or increased in most patients, giving the false impression of preserved systolic function. However, longitudinal strain assessment invariably shows a global and regional decrease (coincident with sites of greater hypertrophy) of contractility. 77 Estimating the size of the LA is fundamental, as there is a significant correlation between the dilation of the chamber and an increased risk of cardiovascular events, such as atrial fibrillation and sudden death. The main complication of HCM is sudden cardiac death (SCD), especially in young and apparently healthy individuals. 78,79 Cardiac defibrillator implantation for primary or secondary prophylaxis may reduce mortality from this complication and is the only therapy with evidence of life- saving potential. 80 TTE has a relevant role in the two most commonly used risk stratification scores form SCD in HCM, which determined the relationship between come clinical risk factors and prognosis. In the American model of primary prevention, one of the risk factors among 5 variables is the presence of interventricular septum thickness ≥ 30 mm. 75 In the European model, of the seven variables analyzed, three of them are provided by TTE: septum thickness, LA and left ventricular outflow tract (LVOT) gradient at rest or after Valsalva maneuver. 72 Family screening of first-degree relatives of HCM subjects should be performed periodically due to their risk of developing the disease. Recommendations for the use of echocardiography in HCM are summarized in table 2. 2.3.7. Restrictive Cardiomyopathies Restrictive cardiomyopathies (RCM) are a group of entities characterized by abnormalities in the ventricular filling pattern, which may be associated with thickened and rigid walls and generally preserved systolic function. RCMs comprise various entities, including, endomyocardial fibrosis (EMF), endomyocardial fibroelastosis, Löefler parietal endocarditis, infiltrative (such as amyloidosis and sarcoidosis), storage (such as hemochromatosis and Fabry disease), idiopathic and other forms secondary to different processes (scleroderma, carcinoid syndrome, metastases of systemic neoplasms, anthracycline toxicity and irradiation heart disease). 81 Diagnosis by echocardiography is based on common anatomical and functional changes: ventricular cavities of normal or reduced size, usually with Doppler degree III diastolic function (restrictive type), generally preserved overall systolic function, and dilated atria. Tissue Doppler analysis shows velocity e’ obtained in the septal mitral annulus usually below 7.0 cm/s, a useful measure in constrictive pericarditis differentiation. 82 In amyloidosis, there is thickening of the atrioventricular valves, myocardial walls, and eventually the atrial septum, with a more intense 145

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