ABC | Volume 111, Nº3, September 2018

Original Article Nemes et al RA function in CA by 3DSTE Arq Bras Cardiol. 2018; 111(3):384-391 of 3DSTE-derived RA assessments has already been confirmed in a recent study. 17 All statistical analyses were carried out using MedCalc software (MedCalc, Inc., Mariakerke, Belgium). Results Two-dimensional Doppler echocardiographic and NT- proBNP data Significantly increased left atrial diameter, interventricular septum (IVS) and left ventricular (LV) posterior wall could be demonstrated inAL-CApatients as compared tomatched controls (Table 1). Significant differences could be detected between AL-CA patients and matched controls in tricuspid annular plane systolic excursion (16.7±3.1mm vs. 20.0±1.8mm, p=0.05) and RV fractional area change (32.3 ± 5.3% vs. 39.2 ± 3.5%, p=0.04). Significant (≥grade 3) mitral regurgitation could not be detected in any of the patients or control subjects. Only 1 patient with AL-CA had grade 4 tricuspid regurgitation. NT-proBNP level proved to be 9983 ± 11101 U/l in AL-CA patients. Three-dimensional speckle tracking echocardiographic data Significant differences could be demonstrated in all RA volumes respecting the cardiac cycle. Total and active atrial emptying fractions were significantly decreased in AL-CA patients, while RA stroke volumes did not differ between the groups examined (Table 2). Peak global and mean segmental area strains proved to be reduced in AL-CA patients as compared to that of matched controls. Midatrial segmental circumferential, longitudinal and area strains, together with some basal strains, proved to be reduced in patients with AL-CA (Tables 3-4). Global longitudinal and area strains at atrial contraction were impaired in AL-CA patients, together with midatrial segmental circumferential and area strains (Tables 5-6). These results could suggest impaired longitudinal and circumferential RA function in the reservoir and active contraction phases of the RA function. Alterations in segmental RA strains could suggest non-uniformity of RA dysfunction in these cases. Discussion Among several types of amyloidosis, in AL amyloidosis is characterized by fibril deposits, which are composed of monoclonal immunoglobulin light chains and is mainly associated with B-cell type diseases, like clonal plasma cell or other B-cell dyscrasias. 18 The course of the disease can be progressive in case of cardiac involvement .19 The main cause of death in patients with AL amyloidosis is cardiac involvement leading to heart failure or arrhythmias, and is considered to be an important prognostic factor. Without cardiac presentation, the survival is 4 years, 20 in some cases, it is only 8 months. 21 In case of cardiac involvement, typically concentric ventricular thickening with RV involvement, biventricular function with normal or near normal ejection fraction and valvular thickening can be seen. 22,23 The speckled or granular myocardial appearance is characteristic of amyloid deposit, but the absence of this phenomenon is not rare. 2 Disproportionate septal deposition can mimic hypertrophic cardiomyopathy with dynamic LV outflow tract obstruction. Atrial thrombus is common, especially in AL-CA, and sometimes is associated to atrial fibrillation. Diastolic dysfunction is the earliest echocardiographic sign and can often be detected before any clinical symptom. 24,25 The end-diastolic thickness of the IVS is > 12 mm in the absence of any other cause of LV hypertrophy in heart involvement. 13 In CA, the thickness of the LV wall is not in correlation with the course and outcome of Table 2 – Comparison of 3DSTE-derived volumetric and volume-based functional right atrial parameters in patients with cardiac amyloidosis and in matched controls AL-CA patients (n = 16) Controls (n = 15) p-value Calculated Volumes Vmax (ml) 85.0 ± 40.2 43.0 ± 13.2 < 0.0001 * Vmin (ml) 69.8 ± 37.3 30.8 ± 9.2 < 0.0001 * VpreA (ml) 79.2 ± 41.0 38.2 ± 12.8 < 0.0001 * Stroke Volumes TASV (ml) 15.2 ± 9.2 12.2 ± 7.3 0.40 * PASV (ml) 5.8 ± 5.1 4.8 ± 3.1 0.98 * AASV (ml) 9.4 ± 8.6 7.4 ± 5.9 0.47 * Emptying fractions TAEF (%) 19.2 ± 9.3 27.9 ± 10.7 0.02 * PAEF (%) 7.9 ± 8.0 11.5 ± 6.8 0.07 * AAEF (%) 12.1 ± 8.1 18.6 ± 9.8 0.05 * 3DSTE: three-dimensional speckle-tracking echocardiography; CA: cardiac amyloidosis; AAEF: active atrial emptying fraction; AASV: active atrial stroke volume; PAEF: passive atrial emptying fraction; PASV: passive atrial stroke volume; TAEF: total atrial emptying fraction; TASV: total atrial stroke volume; Vmax: maximum right atrial volume; Vmin: minimum right atrial volume; VpreA: right atrial volume before atrial contraction. Data expressed as mean ± standard deviation. * Unpaired Student t test. 387