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      Distinguishing Cardiac Amyloidosis and Hypertrophic Cardiomyopathy by Thickness and Myocardial Deformation of the Right Ventricle

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          Abstract

          Objectives

          To compare right ventricular thickness (RVT) and deformation of cardiac amyloidosis (CA) and hypertrophic cardiomyopathy (HCM) patients.

          Methods

          Sixty CA (mean age 58 ± 10 years; 33 males (55%)) and sixty HCM patients (mean age 55 ± 14 years; 27 males (45%)) were retrospectively enrolled. RVT, global radical peak strain (GRPS), global longitudinal peak strain (GLPS), and global circumferential peak stain (GCPS) were analyzed. To determine the cutoff values of the RVT and RV strain parameters for distinguishing CA from HCM, the areas under the receiver operating characteristic curve (AUCs) were analyzed.

          Results

          RVT of CA patients was significantly thicker than that of HCM patients (7.8 ± 2.1 vs 5.9 ± 1.3, p < 0.001). Moreover, significantly decreased RV-GRPS (12.1 ± 6.9 vs 23.5 ± 12.1, p < 0.001), RV-GCPS (−3.4 ± 2.2 vs −5.6 ± 3.5, p < 0.001), and RV-GLPS (−4.6 ± 2.3 vs −11.1 ± 4.9, p < 0.001) were observed in CA patients compared with HCM patients. RVT and RV strain demonstrate comparable diagnostic accuracy in differentiating CA from HCM. In particular, RV-GLPS combined with RVT showed the best performance for discriminating CA from HCM (AUC = 0.92, 95% CI: 0.85 to 0.96, p = 0.0001).

          Conclusions

          Right ventricular myocardial thickness and deformation of CA patients was more severe than HCM patients. RV-GLPS combined with RVT presents an excellent diagnostic performance in distinguishing CA and HCM.

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          Most cited references25

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          Comparing the Areas under Two or More Correlated Receiver Operating Characteristic Curves: A Nonparametric Approach

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            2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC).

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              Hypertrophic cardiomyopathy.

              Hypertrophic cardiomyopathy is a common inherited cardiovascular disease present in one in 500 of the general population. It is caused by more than 1400 mutations in 11 or more genes encoding proteins of the cardiac sarcomere. Although hypertrophic cardiomyopathy is the most frequent cause of sudden death in young people (including trained athletes), and can lead to functional disability from heart failure and stroke, the majority of affected individuals probably remain undiagnosed and many do not experience greatly reduced life expectancy or substantial symptoms. Clinical diagnosis is based on otherwise unexplained left-ventricular hypertrophy identified by echocardiography or cardiovascular MRI. While presenting with a heterogeneous clinical profile and complex pathophysiology, effective treatment strategies are available, including implantable defibrillators to prevent sudden death, drugs and surgical myectomy (or, alternatively, alcohol septal ablation) for relief of outflow obstruction and symptoms of heart failure, and pharmacological strategies (and possibly radiofrequency ablation) to control atrial fibrillation and prevent embolic stroke. A subgroup of patients with genetic mutations but without left-ventricular hypertrophy has emerged, with unresolved natural history. Now, after more than 50 years, hypertrophic cardiomyopathy has been transformed from a rare and largely untreatable disorder to a common genetic disease with management strategies that permit realistic aspirations for restored quality of life and advanced longevity. Copyright © 2013 Elsevier Ltd. All rights reserved.
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                Author and article information

                Contributors
                Journal
                Cardiol Res Pract
                Cardiol Res Pract
                crp
                Cardiology Research and Practice
                Hindawi
                2090-8016
                2090-0597
                2022
                1 February 2022
                : 2022
                : 4364279
                Affiliations
                1Department of Radiology, Second Affiliated Hospital of Nanchang University, Nanchang 330000, China
                2Department of Forensic Genetics, West China School of Basic Medical Sciences & Forensic Medicine, Sichuan University, Chengdu 610041, Sichuan, China
                3Department of Radiology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education; West China Second University Hospital, Sichuan University, Chengdu, Sichuan 610041, China
                4Department of Radiology, National Key Laboratory of Biotherapy, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, China
                5MR Collaboration, Siemens Healthcare Ltd., Shanghai, China
                Author notes

                Academic Editor: Michael Spartalis

                Author information
                https://orcid.org/0000-0001-9791-9950
                https://orcid.org/0000-0002-2326-3423
                https://orcid.org/0000-0001-8437-9887
                Article
                10.1155/2022/4364279
                8825671
                91ae8eac-af34-4feb-94f0-ce0632da7dcf
                Copyright © 2022 Hui Liu et al.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 26 April 2021
                : 3 January 2022
                : 4 January 2022
                Funding
                Funded by: National Natural Science Foundation of China
                Award ID: 82120108015
                Funded by: Sichuan Science and Technology Program
                Award ID: 2020YFS0050
                Award ID: 2020YJ0029
                Award ID: 21ZDYF1967
                Funded by: Fundamental Research Funds for the Central Universities
                Award ID: SCU2020D4132
                Categories
                Research Article

                Cardiovascular Medicine
                Cardiovascular Medicine

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