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 3 – Recommendations of echocardiography in restrictive cardiomyopathies Recommendation Class of recommendation Level of evidence Diagnostic investigation of patients with heart failure without clear etiology I C Differential diagnosis of patients with restrictive syndrome I C Symptomatic patients with systemic diseases potentially causing RCM I C Patients with hypereosinophilic syndrome, ascites and distended jugular veins I C Patients with ascites and lower limb edema, without established diagnosis I C Patients submitted to radiotherapy with signs of systemic venous hypertension I C Reassessment of patients with previous RCM diagnosis when there is a change in the clinical course of the disease I C Patients with EMF for therapeutic planning and prognostic evaluation IIa C Patients with edema and ascites, with evidence of normal systemic venous pressure and no evidence of cardiopathy III C RCM: restrictive cardiomyopathies; EMF: endomyocardial fibrosis. contractile abnormalities and/or dilation and right ventricular dysfunction. Among the traditional echocardiographic criteria, derived from 2D echocardiography, proposed for the diagnosis of AC, 88 are: presence of akinesia, dyskinesia or right ventricular aneurysm; increased ventricular outflow tract diameter (measured on long and short parasternal axis); and reduction of the fractional variation of the RV area. Recently, the routine and systematic addition of other echocardiographic measurements and techniques was recommended in order to improve evaluation: 89 • Conventional parameters: basal RV diameter (normal ≤ 41 mm); systolic excursion of the annular tricuspid plane (TAPSE – normal ≥ 17 mm). • Advanced parameters: wave s’ to the tissue Doppler of the RV’s free wall (normal ≥ 9.5 cm/s); longitudinal strain of the RV’s free wall (normal ≥ -20%); LV’s GLS (normal ≥ -18%); RV ejection fraction to 3D echocardiography (normal ≥ 45%). In short, TTE, preferably with analysis of conventional and advanced parameters, is indicated in patients with suspected or established AC (evaluation of disease progression), as well as in family screening of first-degree relatives. 2.3.9. Non-compaction Cardiomyopathy Non-compaction cardiomyopathy (NCC) is considered a distinct cardiomyopathy, marked by genetic heterogeneity, with an overlapping of different phenotypes and great Table 4 – Recommendations of the echocardiography in arrhythmogenic cardiomyopathy Recommendation Class of recommendation Level of evidence Assessment of patients with suspected AC I B Reassessment of patients with known AC when there is change of symptoms or new cardiovascular event I C Family screening in first-degree relatives of AC patients I C Routine re-evaluation of clinically stable patients with no changes in therapy III C AC: arrhythmogenic cardiomyopathy. variability of clinical presentation. As a consequence, there is still controversy in the literature regarding its nomenclature: whereas for the American Heart Association (AHA) 90 it is considered a primary cardiomyopathy, the European Society of Cardiology (ESC) 81 considers it an unclassified disorder. Its pathogenesis implies early interruption of compaction of the trabecular meshwork of the LV during embryogenesis, resulting in the formation of two layers: a thin compacted epicardial layer and a thick endocardial one (similar to a “spongy” mesh) with marked trabeculations and deep intertrabecular recesses. The 2D echocardiography is the basis for diagnosis, follow-up, and better delineation of NCC phenotypic expressions. 91 Several criteria have been employed in diagnosis, taking into account the increase in the proportion of the non compacted layer (for example, the non compacted/compacted ratio at the end of systole > 2), presence of excessive trabeculation, hypokinesia of non compacted areas (commonly located at the apex and lateral wall) and visualization of flow in the recess (via color Doppler). New techniques have recently been incorporated to aid in diagnosis, such as the use of echocardiographic contrast, 3D echocardiography and myocardial strain for the analysis of regional deformation and rotation (which assumes a characteristic pattern in this nosological entity). 92 Therefore, it should be noted that the diagnosis of suspected cases has increased in recent years, due to advances and improvements in imaging methods, as well as the 147