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      A meta-analysis for the echocardiographic assessment of right ventricular structure and function in ARVC: a Study by the Research and Audit Committee of the British Society of Echocardiography

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          Abstract

          Introduction

          Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited pathology that can increase the risk of sudden death. Current task force criteria for echocardiographic diagnosis do not include new, regional assessment tools which may be relevant in a phenotypically diverse disease. We adopted a systematic review and meta-analysis approach to highlight echocardiographic indices that differentiated ARVC patients and healthy controls.

          Methods

          Data was extracted and analysed from prospective trials that employed a case–control design meeting strict inclusion and exclusion as well as a priori quality criteria. Structural indices included proximal RV outflow tract (RVOT 1) and RV diastolic area (RVD area). Functional indices included RV fractional area change (RVFAC), tricuspid annular systolic excursion (TAPSE), peak systolic and early diastolic myocardial velocities (S′ and E′, respectively) and myocardial strain.

          Results

          Patients with ARVC had larger RVOT 1 (mean ±  s.d.; 34 vs 28 mm, P < 0.001) and RVD area (23 vs 18 cm 2, P < 0.001) compared with healthy controls. ARVC patients also had lower RVFAC (38 vs 46%, P < 0.001), TAPSE (17 vs 23 mm, P < 0.001), S′ (9 vs 12 cm/s, P < 0.001), E′ (9 vs 13 cm/s, P < 0.001) and myocardial strain (−17 vs −30%, P < 0.001).

          Conclusion

          The data from this meta-analysis support current task force criteria for the diagnosis of ARVC. In addition, other RV measures that reflect the complex geometry and function in ARVC clearly differentiated between ARVC and healthy controls and may provide additional diagnostic and management value. We recommend that future working groups consider this data when proposing new/revised criteria for the echocardiographic diagnosis of ARVC.

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          Most cited references35

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          Right ventricular function and failure: report of a National Heart, Lung, and Blood Institute working group on cellular and molecular mechanisms of right heart failure.

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            Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology.

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              Arrhythmogenic Right Ventricular Cardiomyopathy: Dysplasia, Dystrophy, or Myocarditis?

              Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a frequent cause of sudden death in young individuals and athletes. Although familial occurrence has been documented and a gene defect was recently localized on chromosome 14q23-q24 the etiopathogenesis of the disease is still obscure. A pathological study was conducted in 30 hearts with ARVC (age range, 15 to 65 years; mean, 28 years). In the 27 autopsy cases, the mode of death was sudden in 24 and congestive heart failure in 3. ECG, available in 19 cases, showed inverted T waves in the right precordial leads in 15 cases (79%) and ventricular arrhythmias in 15 (79%). Right ventricular aneurysms were present in 15 hearts (50%) and located in the inferior wall in 12. Left ventricle and ventricular septum were involved in 14 (47%) and 6 (20%) cases, respectively. Scattered foci of lymphocytes with myocardial death were observed in 20 cases (67%). Electron microscopy studies, although confirming the myocardial death and lymphocyte infiltrates, did not show any specific ultrastructural substrate. Two pathological patterns, fatty (40%) and fibrofatty (60%), were identified. The fibrofatty pattern was associated with a thinner right ventricular wall (P < .0001) and a higher occurrence of focal myocarditis (P < .001). In sections of right ventricular free wall with maximal fatty infiltration, the mean percentage area of fatty tissue was 35.9 +/- 11.1% in control versus 80.4 +/- 9.6% in the ARVC, fatty variety (P < .00001). Involvement of the left ventricle and/or ventricular septum, right ventricular aneurysms, and inflammation were found almost exclusively in the fibrofatty variety. In the fibrofatty variety of ARVC, the myocardial atrophy appears to be the consequence of acquired injury (myocyte death) and repair (fibrofatty replacement), mediated by patchy myocarditis. Whether the inflammation is a primary event or a reaction to spontaneous cell death remains unclear.
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                Author and article information

                Journal
                Echo Res Pract
                Echo Res Pract
                echo
                Echo Research and Practice
                Bioscientifica Ltd (Bristol )
                2055-0464
                September 2016
                September 2016
                : 3
                : 3
                : 95-104
                Affiliations
                [1 ]Research Institute for Sport and Exercise Sciences , Liverpool John Moores University, Liverpool, UK
                [2 ]Countess of Chester Hospital , NHS Trust, Chester, UK
                [3 ]Barts Heart Centre , St Bartholomew’s Hospital, London, UK
                [4 ]Papworth Hospital NHS Trust , Cambridge, UK
                [5 ]West Suffolk NHS Trust , Bury St Edmonds, UK
                [6 ]National Heart and Lung Institute , Imperial College, London, UK
                [7 ]St Georges University Hospital , London, UK
                [8 ]Guys and St Thomas’s NHS Trust , London, UK
                [9 ]Leeds Teaching Hospitals NHS Trust , Leeds, UK
                [10 ]University Hospitals Birmingham NHS Trust and Institute of Cardiovascular Sciences , University of Birmingham, Birmingham, UK
                [11 ]Department of Cardiology , University Hospital of South Manchester, Manchester, UK
                [12 ]Royal United Hospital , Bath, UK
                Author notes
                Correspondence should be addressed to D Oxborough; Email: d.l.oxborough@ 123456ljmu.ac.uk
                Article
                ERP160028
                10.1530/ERP-16-0028
                5076568
                27686556
                5174ecd4-8661-4f68-ba0f-904ee2101835
                © 2016 The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : 27 September 2016
                : 29 September 2016
                Categories
                Research

                echocardiography,arrhythmogenic right ventricular cardiomyopathy,arvc

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