ABC | Volume 113, Nº1, July 2019

Statement Position Statement on Indications of Echocardiography in Adults – 2019 Arq Bras Cardiol. 2019; 113(1):135-181 Table 2 – Recommendations of transthoracic echocardiography, echocardiography under physical stress and transesophageal echocardiography in hypertrophic cardiomyopathy 72,75 Recommendation Class of recommendation Level of evidence TTE in the initial assessment of all patients with suspected HCM, at rest and during Valsalva maneuver I B EPS in symptomatic patients with resting or Valsalva intraventricular gradient < 50 mmHg to assess the degree of dynamic obstruction and mitral regurgitation during exercise I B Reassessment by TTE when there are changes in symptoms or a new cardiovascular event I B TTE in the assessment of therapeutic results of pharmacological, surgical (myomectomy), interventional (alcoholic septal artery occlusion) and pacemaker treatments I C TTE in the screening of first-degree relatives with HCM diagnosis I B Serial TTE (every 12 to 18 months) in children of HCM patients, starting at age 12 (or earlier, if there is intention to take on competitive sports or sudden death among relatives) I C TTE during alcohol septal artery ablation I B TEE in intraoperative myectomy monitoring and during alcoholic occlusion of the septal artery with inadequate TTE I B Serial TTE every one to two years may be useful in stable symptomatic patients to reassess myocardial hypertrophy, dynamic obstruction, and ventricular function IIa C TEE may be useful when TTE is inconclusive, in the planning of myomectomy or in the evaluation of mitral regurgitation secondary to mitral valve abnormalities IIa C Serial TTE (every five years) is reasonable in periodic reassessment in first-degree relatives of adult HCM patients IIa C TTE combined with intravenous contrast injection is reasonable if the diagnosis of apical HCM and/ or apical infarction are doubtful, or the quantification of hypertrophy is inadequate, especially if MRI is unavailable, non-diagnostic or contraindicated IIa C EPS may be useful in asymptomatic HC patients, with no dynamic obstruction at rest when gradient detection in LVOT is relevant for lifestyle or career change orientation, or decision making on medical treatment IIb C TTE should not be performed in less than 12 months in HCM patients when there is no change in symptoms or predicted conduct change III C TTE: transthoracic echocardiography; HCM: hypertrophic cardiomyopathy; EPS: echocardiography under physical stress; TEE: transesophageal echocardiography; LVOT: left ventricular outflow tract. (ecorrefringence) reflection and “granular and sparkling” myocardial aspect. 83 The GLS analysis of the LV with 2D echocardiography in amyloidosis observes very low values, especially in the mid and basal segments with relative “apical sparing” (it aids in the differential diagnosis with other diseases). 84 In EMF, one may observe: obliteration of the apex by fibrosis, signs of ventricular restriction, and involvement of the atrioventricular valves. The fibrosis of the apical thrombi is differentiated due to absence of akinesia or dyskinesia in the left EMF. Another differential diagnosis is apical HCM, which presents no endocardial thickening or restrictive pattern and displays specific electrocardiographic changes. Cardiac sarcoidosis may present with regional contractile abnormalities and non-ischemic distribution aneurysms. GLS measure represents an early marker of myocardial involvement in sarcoidosis and the magnitude of the reduction is associated with poor prognosis. 85 The recommendations for TTE in RCM are set out in table 3. Transesophageal echocardiography (TEE) is indicated when there are technical difficulties to TTE and in the transoperative monitoring of fibrosis and apical correction of valve defects. 2.3.8. Arrhythmogenic Cardiomyopathy (Right Ventricle Arrhythmogenic Dysplasia) Arrhythmogenic cardiomyopathy (AC) is considered an inherited cardiomyopathy with autosomal dominant transmission, predisposing to the emergence of ventricular arrythmias, sudden death in young people, ventricular dysfunction and heart failure. Due to the frequent involvement of the LV, the use of the term CA is currently recommended, which comprises both ventricles, replacing the term “arrhythmogenic dysplasia of the RV”. 86 The disease is characterized by a progressive replacement of the ventricular myocardium by fibrous and adipose tissue, which can lead to thinning of the wall and aneurysm formation. In the RV, the process is typically located in the inferior, apical and infundibular walls (dysplasia triangle), and may be diffuse or segmental. LV involvement occurs in more than half of the cases, typically located in the subepicardium or mesocardium, and often confined to the inferolateral segment. Echocardiography is the imaging modality of choice in the initial assessment of AC (Table 4) and the most commonly used propaedeutic method for patient follow-up. 87 Typical morphologic features in patients with CA include regional 146