ABC | Volume 111, Nº3, September 2018

Original Article Right Atrial Deformation Analysis in Cardiac Amyloidosis – Results from the Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study Attila Nemes, Dóra Földeák, Péter Domsik, Anita Kalapos, Árpád Kormányos, Zita Borbényi, Tamás Forster 2nd Department of Medicine and Cardiology Center, Medical Faculty, Albert Szent-Györgyi Clinical Center, University of Szeged, Szeged - Hungary Mailing Address: Attila Nemes • Semmelweis street 8. 6725, Szeged – Hungary E-mail: nemes@in2nd.szote.u-szeged.hu , attila.nemes.md@gmail.com Manuscript received October 02, 2017, revised manuscript April 11, 2018, accepted April 11, 2018 DOI: 10.5935/abc.20180150 Abstract Background: Light-chain (AL) cardiac amyloidosis (CA) is characterized by fibril deposits, which are composed of monoclonal immunoglobulin light chains. The right ventricle is mostly involved in AL-CA and impairment of its function is a predictor of worse prognosis. Objectives: To characterize the volumetric and functional properties of the right atrium (RA) in AL-CA by three‑dimensional speckle-tracking echocardiography (3DSTE). Methods: A total of 16 patients (mean age: 64.5±10.1 years, 11males) with AL-CAwere examined. Their results were compared to that of 15 age- and gender-matched healthy controls (mean age: 58.9 ± 6.9 years, 8 males). All cases have undergone complete two-dimensional Doppler and 3DSTE. A two-tailed p value of less than 0.05 was considered statistically significant. Results: Significant differences could be demonstrated in RA volumes respecting cardiac cycle. Total (19.2 ± 9.3% vs. 27.9 ± 10.7%, p = 0.02) and active atrial emptying fractions (12.1 ± 8.1 vs. 18.6 ± 9.8%, p = 0.05) were significantly decreased in AL-CA patients. Peak global (16.7 ± 10.3% vs. 31.2 ± 19.4%, p = 0.01) and mean segmental (24.3 ± 11.1% vs. 38.6 ± 17.6%, p =0.01) RA area strains, together with some circumferential, longitudinal and segmental area strain parameters, proved to be reduced in patients with AL-CA. Global longitudinal (4.0 ± 5.2% vs. 8.2 ± 5.5%, p = 0.02) and area (7.8 ± 8.1% vs. 15.9 ± 10.3%, p = 0.03) strains at atrial contraction and some circumferential and area strain parameters at atrial contraction were reduced in AL-CA patients. Conclusion: Significantly increased RA volumes and deteriorated RA functions could be demonstrated in AL-CA. (Arq Bras Cardiol. 2018; 111(3):384-391) Keywords: Amyloidosis; Echocardiography, Three Dimensional / methods; Humans; Ventricular Dysfunction, Right; Speckle-Tracking. Introduction Systemic amyloidosis is a rare disease caused by the extracellular deposition of protein (amyloid) fibrils, which are composed of low molecular weight subunits (5 to 25 kD) of various serum proteins. 1 The amyloid fibrils progressively damage the structure and function of the affected tissue, with variable clinical symptoms. 2,3 For diagnosis of amyloidosis, biopsy from the affected tissue or from (abdominal) subcutaneous adipose tissue is necessary in most of cases. 4 The classification of amyloidosis depends on the type of the precursor protein, including acquired monoclonal immunoglobulin light-chain amyloidosis (AL), wild-type or hereditary transthyretin amyloidosis (TTR), acquired serum amyloid type (AA) and other rare types. The mortality is especially high in light-chain (AL) amyloidosis. 5,6 There are some warning signs that can draw attention to amyloidosis, such as nephrotic syndrome, tissue infiltration such as macroglossia, respiratory disease, carpal tunnel syndrome, bleeding, cachexia, haematological disease such as multiple myeloma, and genetic predisposition. As for the clinical signs, syncope is a poor prognostic factor and occurs quite frequently in patients with cardiac involvement. 7 Cardiac involvement in amyloidosis varies according to the type of the disease. 1 The real incidence of cardiac amyloidosis (CA) is not known precisely and is often diagnosed only during autopsy. 8 Heart failure usually occurs in CA due to the combination of decreased myocardial compliance and compressed myocardial cells. These changes develop due to the infiltration by amyloid deposits and could lead to restrictive cardiomyopathy. 1 Arrhythmias, pleural and pericardial effusion can also be detected in some cases. 4,9,10 Although the right ventricle (RV) is mostly involved in CA, limited data is available about the involvement of the right atrium (RA). 11,12 Therefore, this study aimed to characterize the volumetric and functional properties of the RA in AL-CA by three‑dimensional (3D) speckle-tracking echocardiography (3DSTE). 384