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1

Markus, Frank I., Andrea Nava, and Gaetano Thiene, eds. Arrhythmogenic RV Cardiomyopathy/Dysplasia. Springer Milan, 2007. http://dx.doi.org/10.1007/978-88-470-0490-0.

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2

Andrea, Nava, Rossi Lino, and Thiene Gaetano, eds. Arrhythmogenic right ventricular cardiomyopathy/dysplasia: Proceedings of the 1st International Symposium on [T.B.A.]. Elsevier, 1997.

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3

Harmon, Jordan, Tufts-New England Medical Center. Evidence-based Practice Center., and United States. Agency for Healthcare Research and Quality., eds. Effects of omega-3 fatty acids on arrhythmogenic mechanisms in animal and isolated organ/cell culture studies. Agency for Healthcare Research and Quality, U.S. Dept. of Health and Human Services, 2004.

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4

United States. Agency for Healthcare Research and Quality, ed. Effects of omega-3 fatty acids on arrhythmogenic mechanisms in animal and isolated organ/cell culture studies. Agency for Healthcare Research and Quality, 2004.

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5

United States. Agency for Healthcare Research and Quality., ed. Effects of omega-3 fatty acids on arrhythmogenic mechanisms in animal and isolated organ/cell culture studies. Agency for Healthcare Research and Quality, 2004.

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6

Harmon, Jordan, Tufts-New England Medical Center. Evidence-based Practice Center, and United States. Agency for Healthcare Research and Quality, eds. Effects of omega-3 fatty acids on arrhythmogenic mechanisms in animal and isolated organ/cell culture studies. Agency for Healthcare Research and Quality, U.S. Dept. of Health and Human Services, 2004.

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7

Harmon, Jordan, Tufts-New England Medical Center. Evidence-based Practice Center, and United States. Agency for Healthcare Research and Quality, eds. Effects of omega-3 fatty acids on arrhythmogenic mechanisms in animal and isolated organ/cell culture studies. Agency for Healthcare Research and Quality, U.S. Dept. of Health and Human Services, 2004.

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8

Harmon, Jordan, Tufts-New England Medical Center. Evidence-based Practice Center., and United States. Agency for Healthcare Research and Quality., eds. Effects of omega-3 fatty acids on arrhythmogenic mechanisms in animal and isolated organ/cell culture studies. Agency for Healthcare Research and Quality, U.S. Dept. of Health and Human Services, 2004.

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9

Harmon, Jordan, Tufts-New England Medical Center. Evidence-based Practice Center., and United States. Agency for Healthcare Research and Quality., eds. Effects of omega-3 fatty acids on arrhythmogenic mechanisms in animal and isolated organ/cell culture studies. Agency for Healthcare Research and Quality, U.S. Dept. of Health and Human Services, 2004.

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10

United States. Agency for Healthcare Research and Quality., ed. Effects of omega-3 fatty acids on arrhythmogenic mechanisms in animal and isolated organ/cell culture studies. Agency for Healthcare Research and Quality, 2004.

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11

Harmon, Jordan, United States. Agency for Healthcare Research and Quality., and New England Medical Center Hospital. Evidence-based Practice Center., eds. Effects of omega-3 fatty acids on arrhythmogenic mechanisms in animal and isolated organ/cell culture studies. U.S. Dept. of Health and Human Services, Public Health Service, Agency for Healthcare Research and Quality, 2004.

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12

United States. Agency for Healthcare Research and Quality. Effects of omega-3 fatty acids on arrhythmogenic mechanisms in animal and isolated organ/cell culture studies. Agency for Healthcare Research and Quality, U.S. Department of Health and Human Services, 2004.

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13

United States. Agency for Healthcare Research and Quality, ed. Effects of omega-3 fatty acids on arrhythmogenic mechanisms in animal and isolated organ/cell culture studies. Agency for Healthcare Research and Quality, 2004.

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14

United States. Agency for Healthcare Research and Quality., ed. Effects of omega-3 fatty acids on arrhythmogenic mechanisms in animal and isolated organ/cell culture studies. Agency for Healthcare Research and Quality, 2004.

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15

United States. Agency for Healthcare Research and Quality, ed. Effects of omega-3 fatty acids on arrhythmogenic mechanisms in animal and isolated organ/cell culture studies. Agency for Healthcare Research and Quality, 2004.

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16

Harmon, Jordan, Tufts-New England Medical Center. Evidence-based Practice Center, and United States. Agency for Healthcare Research and Quality, eds. Effects of omega-3 fatty acids on arrhythmogenic mechanisms in animal and isolated organ/cell culture studies. Agency for Healthcare Research and Quality, U.S. Dept. of Health and Human Services, 2004.

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17

Hariharan, Venkatesh. The Effects of Arrhythmogenic Right Ventricular Cardiomyopathy-Causing Proteins on the Mechanical and Signaling Properties of Cardiac Myocytes. [publisher not identified], 2014.

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18

Syrris, Petros, and Alexandros Protonotarios. Arrhythmogenic right ventricular cardiomyopathy: genetics. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780198784906.003.0359.

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Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disorder of the heart muscle which is typically inherited in an autosomal dominant manner. It is believed to be familial in over 50% of cases. A recessive mode of inheritance has also been reported in syndromic cases with cardiocutaneous features. The classic form of the disorder is considered to be ‘a disease of the desmosome’ as pathogenic variants have been identified in five genes encoding key desmosomal proteins: plakoglobin, desmoplakin, plakophilin-2, desmoglein-2, and desmocollin-2. Mutations in these genes account for 30–50%
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19

Bass, Cristina, Barbara Bauce, and Gaetano Thiene. Arrhythmogenic right ventricular cardiomyopathy: diagnosis. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780198784906.003.0360.

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Arrhythmogenic cardiomyopathy is a heart muscle disease clinically characterized by life-threatening ventricular arrhythmias and pathologically by an acquired and progressive dystrophy of the ventricular myocardium with fibrofatty replacement. The clinical manifestations of arrhythmogenic cardiomyopathy vary according to the ‘phenotypic’ stage of the underlying disease process. Since there is no ‘gold standard’ to reach the diagnosis of arrhythmogenic cardiomyopathy, multiple categories of diagnostic information have been combined. Different diagnostic categories include right ventricular morp
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20

Katritsis, Demosthenes G., Bernard J. Gersh, and A. John Camm. Arrhythmogenic right ventricular cardiomyopathy/dysplasia. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199685288.003.0918_update_004.

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21

Touboul, Paul, and Marjaneh Fatemi. Touboul Arrhythmogenic Right Ventricular Cardiomyopathy. Wiley & Sons, Incorporated, John, 2008.

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22

Marcus, Frank I., Andrea Nava, and Gaetano Thiene. Arrhythmogenic RV Cardiomyopathy/Dysplasia: Recent Advances. Springer London, Limited, 2007.

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23

Marcus, Frank I., Andrea Nava, and Gaetano Thiene. Arrhythmogenic RV Cardiomyopathy/Dysplasia: Recent Advances. Springer, 2014.

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24

Elliott, Perry, Kristina H. Haugaa, Pio Caso, and Maja Cikes. Restrictive cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780198726012.003.0044.

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Restrictive cardiomyopathy is a heart muscle disorder characterized by increased myocardial stiffness that results in an abnormally steep rise in intraventricular pressure with small increases in volume in the presence of normal or decreased diastolic left ventricular volumes and normal ventricular wall thickness. The disease may be caused by mutations in a number of genes or myocardial infiltration. Arrhythmogenic right ventricular cardiomyopathy is an inherited cardiac muscle disease associated with sudden cardiac death, ventricular arrhythmias, and cardiac failure. It is most frequently cau
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25

(Editor), Frank I. Marcus, Andrea Nava (Editor), and Gaetano Thiene (Editor), eds. Arrhythmogenic right ventricular cardiomyopathy/dysplasia: Recent Advances. Springer, 2007.

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26

Brunckhorst, Corinna, Firat Duru, and Ardan M. Saguner. Current Concepts in Arrhythmogenic Cardiomyopathy, Second Edition. Cardiotext Publishing, 2021.

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27

Brunckhorst, Corrina. Current Concepts in Arrhythmogenic Cardiomyopathy, Second Edition. Cardiotext Publishing, 2021.

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28

Brunckhorst, Corinna, Firat Duru, and Ardan M. Saguner. Current Concepts in Arrhythmogenic Right Ventricular Cardiomyopathy / Dysplasia. Cardiotext Publishing, 2014.

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29

PhD, Domenico Corrado MD, Gaetano Thiene MD, and Cristina Basso MD. Arrhythmogenic Cardiomyopathy, An Issue of Cardiac Electrophysiology Clinics. Saunders, 2011.

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30

Current Concepts in Arrhythmogenic Right Ventricular Cardiomyopathy / Dysplasia. Cardiotext Publishing, 2014.

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31

(Editor), John A. Camm, ed. Arrhythmogenic Right Ventricular Cardiomyopathy (Clinical Approaches to Tachyarrhythmias, V. 17). Blackwell Publishing Limited, 2002.

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32

Elliott, Perry, and Alexandros Protonotarios. Arrhythmogenic right ventricular cardiomyopathy: management of symptoms and prevention of sudden cardiac death. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780198784906.003.0361.

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Patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) have arrhythmia-related symptoms or are identified during screening of an affected family. Heart failure symptoms occur late in the disease’s natural history. As strenuous exercise has been associated with disease acceleration and worsening of ventricular arrhythmias, lifestyle modification with restricted athletic activities is recommended upon disease diagnosis or even identification of mutation carrier status. An episode of an haemodynamically unstable, sustained ventricular tachycardia or ventricular fibrillation as well
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33

Cardiac MRI in Diagnosis, Clinical Management, and Prognosis of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia. Elsevier, 2016. http://dx.doi.org/10.1016/c2013-0-19332-4.

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34

Abidov, Aiden, Isabel Oliva, and Frank I. MARCUS. Cardiac MRI in Diagnosis, Clinical Management, and Prognosis of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia. Elsevier Science & Technology Books, 2016.

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35

Nuyens, Dieter. Generation & Characterization of a Mouse Model for a Sodium Channel Based Long Qt Syndrome (Lqt3), an Inherited Arrhythmogenic Disease. Leuven University Press, 2006.

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36

Marcus, Frank I., Andrea Nava, and Gaetano Thiene. ArrhythmogenicRV Cardiomyopathy/Dysplasia. Springer, 2008.

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37

Walkey, Allan J., and Jared Magnani. Therapeutic strategy in tachyarrhythmias. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199600830.003.0156.

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The therapeutic approach to tachyarrhythmias in the critically-ill patient involves rapid diagnosis and haemodynamic assessment. Patients who become haemodynamically unstable due to tachyarrhythmia warrant direct current cardioversion. Effects of direct current cardioversion during critical illness are often transient unless the underlying arrhythmia precipitant is eliminated and longer-acting rate- or rhythm-controlling medications are instituted. Atrial fibrillation often responds favourably to a rate-control strategy and treatment of the underlying precipitants. After initial clinical stabi
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38

Sheppard, Mary N. Myocardial non-compaction. Edited by José Maria Pérez-Pomares, Robert G. Kelly, Maurice van den Hoff, et al. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780198757269.003.0026.

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Isolated left ventricular non-compaction is a controversial entity which has only been reported in the past 30 years. It is becoming more frequently diagnosed due to the use of echocardiography and MRI. It can present in fetal life, infancy, childhood, and adult life. Clinically, the patient can present with cardiac arrhythmias, cardiac failure, systemic emboli due to thrombosis within the ventricles, and sudden death. It can be a genetic entity associated with mutations in many genes associated with hypertrophic cardiomyopathy, dilated cardiomyopathy, and arrhythmogenic cardiomyopathy. It is
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39

Lancellotti, Patrizio, and Bernard Cosyns. Cardiomyopathies. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780198713623.003.0008.

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This chapter focuses on the role of echocardiography in dilated cardiomyopathy, showing diagnostic and associated findings along with the prognostic role of echocardiography. Primary myocardial disease is inadequate hypertrophy, independent of loading conditions and often other affected structures such as mitral valve apparatus, small coronary arteries, and cardiac interstitium. Arrhythmogenic RV cardiomyopathy is fatty or fibro-fatty infiltration of the RV with apoptosis and hypertrophied trabeculae of the RV. This chapter also details diagnostic findings and progression of this condition alo
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40

D’Andrea, Antonello, André La Gerche, and Christine Selton-Suty. Systemic disease and other conditions: athlete’s heart. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780198726012.003.0055.

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The term ‘athlete’s heart’ refers to the structural, functional, and electrical adaptations that occur as a result of habitual exercise training. It is characterized by an increase of the internal chamber dimensions and wall thickness of both atria and ventricles. The athlete’s right ventricle also undergoes structural, functional, and electrical remodelling as a result of intense exercise training. Some research suggests that the haemodynamic stress of intense exercise is greater for the right heart and, as a result, right heart remodelling is slightly more profound when compared with the lef
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41

Rahimi, Kazem. Heart muscle disease (cardiomyopathy). Edited by Patrick Davey and David Sprigings. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199568741.003.0106.

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Cardiomyopathy is defined as disease of heart muscle, and typically refers to diseases of ventricular myocardium. A consensus statement of the European Society of Cardiology (ESC) working group on myocardial and pericardial diseases, published in 2007, abandoned the inconsistent and rather arbitrary classification into primary and secondary causes and based its classification on ventricular morphology and function only. This classification distinguishes five types of cardiomyopathy: dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy, arrhythmogenic right ventricula
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42

Fruhwald, Sonja, and Peter Holzer. Gastrointestinal motility drugs in critical illness. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199600830.003.0040.

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Gastrointestinal motility disturbances in critically-ill patients often require treatment with prokinetic drugs. The aetiology of motility disturbances is complex, and involves electrolyte imbalances, hypervolaemia, reduced intestinal secretion, adverse effects of drugs (catecholamines, opioids, or sedatives) and disease- or treatment-related changes of microflora. However, the choice of prokinetics is narrow, and the multiplicity of pathophysiological mechanisms often limits their efficacy. Gastroparesis can be managed with gastrokinetics such as domperidone, metoclopramide and erythromycin.
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43

Peter, Karlheinz. Regionale Inhomogenitäten des kardialen Erregungsablaufs unter arrhythmogenen Bedingungen und methodische Grenzen ihrer Erfassung durch Monophasische Aktionspotentiale. 1989.

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44

Engelmann, Matthias. Die hochauflösende magnetokardiographie: Eine neue methode zur lokalisierung arrhythmogener zentren und zur detektion pathologischer erregungsausbreitung am herzen. 1993.

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