Das Medizinportal     Aktuelles für medizinische Fachkreise

Das Medizinportal     Aktuelles für medizinische Fachkreise


Entzündliche Kardiomyopathien

Felicitas Escher, CarstenTschöpe, Heinz-Peter Schultheiss, Burkert Pieske


  1. Elliott, P. B. Andersson, E. Arbustini et al. Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases.: Eur Heart J. 2008; 29:270–6.
  2. Caforio AL, Calabrese F, Angeline A et al. A prospective study of biopsy-proven myocarditis: prognostic relevance of clinical and aetiopathogenetic features at diagnosis. Eur Heart J 2007; 28: 1326–33.
  3. Sagar S, Liu PP, Cooper LT. Myocarditis. Lancet 2013; 379:738–747.
  4. Schultheiss HP, Kuehl U, Cooper LT. The Management of Myocarditis. Eur Heart J. 2011; 32:2616–65.
  5. Aretz HT. Myocarditis: the Dallas criteria. Hum. Pathol. 1987; 18(6), 619–624.
  6. Richardson P, McKenna W, Bristow M et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies. Circulation. 1996; 93:841–2.
  7. Caforio AL, Pankuweit S, Arbustini E et al. Current state of knowledge on aetiology, diagnosis, management, and therapy of myocarditis: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J. 2013 Sep; 34(33):2636–48. 
  8. Kühl U, Pauschinger M, Noutsias M et al. High prevalence of viral genomes and multiple viral infections in the myocardium of adults with "idiopathic" left ventricular dysfunction. Circulation. 2005 Feb 22; 111(7):887–93.
  9. Pauschinger M, Bowles NE, Fuentes-Garcia FJ et al. Detection of adenoviral genome in the myocardium of adult patients with idiopathic left ventricular dysfunction. Circulation. 1999 Mar 16; 99(10):1348–54.
  10. Tschöpe C, Bock CT, Kasner M et al. High prevalence of cardiac parvovirus B19 infection in patients with isolated left ventricular diastolic dysfunction. Circulation. 2005 Feb 22; 111(7):879–86. 
  11. Maleszewski JJ, Orellana VM, Hodge DO, Kuhl U, Schultheiss HP, Cooper LT. Long-term risk of recurrence, morbidity and mortality in giant cell myocarditis. Am J Cardiol. 2015 Jun 15; 115(12):1733–8. 
  12. Elezkurtaj S, Lassner D, Schultheiss HP, Escher F. Vascular involvement in cardiac giant cell myocarditis: a new pathophysiological aspect. Clin Res Cardiol. 2014 Feb; 103(2):161–3.
  13. Luk, A., E. Ahn, G. S. Soor et al. Dilated cardiomyopathy: a review: J Clin Pathol. 2009; 62:219–25.
  14. Gorbea C, Makar KA, Pauschinger M et al. A role for Toll-like receptor 3 variants in host susceptibility to enteroviral myocarditis and dilated cardiomyopathy. J Biol Chem. 2010 Jul 23; 285(30):23208–23.
  15. Li, H., D. Ligons, N. Rose. Genetic complexity of autoimmune myocarditis. Autoimmun Rev.2008; 7:168–73.
  16. Leuschner, F., H. A. Katus, and Z. Kaya. Autoimmune myocarditis: past, present and future: J Autoimmun. 2009; 33:282–9.
  17. Pauschinger M, Doerner A, Kuehl U et al. Enteroviral RNA replication in the myocardium of patients with left ventricular dysfunction and clinically suspected myocarditis. Circulation 1999; 99:889–895.
  18. Kühl U et al. miRNA as activity markers in Parvo B19 associated heart disease. Herz. 2012 Sep; 37(6):637–43.
  19. Lindner D, Zietsch C, Tank J et al. Cardiac fibroblasts support cardiac inflammation in heart failure. Basic Res Cardiol. 2014 Sep; 109(5):428.
  20. Escher F, Kühl U, Lassner D et al. Presence of Perforin in Endomyocardial Biopsies of Patients with Inflammatory Cardiomyopathy Predicts Poor Outcome. Eur J Heart Fail. 2014 Oct; 16(10):1066–72.
  21. Escher F, Lassner D, Kühl U et al. Analysis of Endomyocardial Biopsies in suspected Myocarditis - Diagnostic Value on Left versus Right Ventricular Biopsy. International Journal of Cardiology 2014 Nov 15; 177(1):76–78.
  22. Cooper LT, Baughman KL, Feldman AM et al. The role of endomyocardial biopsy in the management of cardiovascular disease: a scientific statement from the American Heart Association, the American College of Cardiology, and the European Society of Cardiology. Endorsed by the Heart Failure Society of America and the Heart Failure Association of the European Society of Cardiology. J Am Coll Cardiol. 2007 Nov 6; 50(19):1914–31. 
  23. Holzmann M, Nicko A, Kühl U et al. Complication rate of right ventricular endomyocardial biopsy via the femoral approach. Circulation 2008; 118: 1722–1728.
  24. Lassner D, Kühl U, Siegismund CD et al. Improved diagnosis of idiopathic giant cell myocarditis and cardiac sarcoidosis by myocardial gene expressing profil. Eur Heart J, 2014 Mar 24.
  25. Kuehl U, Lassner D, von Schlippenbach J et al. Interferon-beta- Improves Survival in Enterovirus-Associated Cardiomyopathy. J Am Coll Cardiol. 2012; 60:1295–6.
  26. Wojnicz R, Nowalany-Kozielska E, Wojciechowska C. Randomized, placebo-controlled study for immunosuppressive treatment of inflammatory dilated cardiomyopathy: two-year follow-up results. Circulation. 2001 Jul 3; 104(1):39–45.
  27. Frustaci A, Russo MA, Chimenti C. Randomized study on the efficacy of immunosuppressive therapy in patients with virus-negative inflammatory cardiomyopathy: the TIMIC study. Eur Heart J. 2009 Aug; 30(16):1995–2002. 

Vererbliche primäre Arrhythmie-Syndrome („Channelopathies“)

Lea Seidlmayer, Fabian Riediger, Oliver Ritter


  1. Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, Berul C, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm Off J Heart Rhythm Soc. 2013 Dec; 10(12):1932–63. 
  2. Schwartz PJ, Stramba-Badiale M, Crotti L, Pedrazzini M, Besana A, Bosi G, et al. Prevalence of the congenital long-QT syndrome. Circulation. 2009 Nov 3; 120(18):1761–7. 
  3. Schwartz PJ, Moss AJ, Vincent GM, Crampton RS. Diagnostic criteria for the long QT syndrome. An update. Circulation. 1993 Aug; 88(2):782–4. 
  4. Moss AJ, Zareba W, Hall WJ, Schwartz PJ, Crampton RS, Benhorin J, et al. Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation. 2000 Feb 15;101(6): 616–23. 
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  7. Napolitano C, Novelli V, Francis MD, Priori SG. Genetic modulators of the phenotype in the long QT syndrome: state of the art and clinical impact. Curr Opin Genet Dev. 2015 Aug 1; 33:17–24. 
  8. Fowler SJ, Priori SG. Clinical spectrum of patients with a Brugada ECG. Curr Opin Cardiol. 2009 Jan; 24(1):74–81. 
  9. Bezzina C, Veldkamp MW, van Den Berg MP, Postma AV, Rook MB, Viersma JW, et al. A single Na(+) channel mutation causing both long-QT and Brugada syndromes. Circ Res. 1999 Dec 3; 85(12):1206–13. 
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  11. Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, Berul C, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm Off J Heart Rhythm Soc. 2013 Dec; 10(12):e85–108. 
  12. Priori SG, Gasparini M, Napolitano C, Della Bella P, Ottonelli AG, Sassone B, et al. Risk stratification in Brugada syndrome: results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry. J Am Coll Cardiol. 2012 Jan 3; 59(1):37–45. 
  13. Haïssaguerre M, Extramiana F, Hocini M, Cauchemez B, Jaïs P, Cabrera JA, et al. Mapping and ablation of ventricular fibrillation associated with long-QT and Brugada syndromes. Circulation. 2003 Aug 26; 108(8):925–8. 
  14. Nademanee K, Veerakul G, Chandanamattha P, Chaothawee L, Ariyachaipanich A, Jirasirirojanakorn K, et al. Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium. Circulation. 2011 Mar 29; 123(12):1270–9. 
  15. Ackerman MJ, Priori SG, Willems S, Berul C, Brugada R, Calkins H, et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies: this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Eur Eur Pacing Arrhythm Card Electrophysiol J Work Groups Card Pacing Arrhythm Card Cell Electrophysiol Eur Soc Cardiol. 2011 Aug; 13(8):1077–109. 
  16. De Ferrari GM, Dusi V, Spazzolini C, Bos JM, Abrams DJ, Berul CI, et al. Clinical Management of Catecholaminergic Polymorphic Ventricular Tachycardia: The Role of Left Cardiac Sympathetic Denervation. Circulation. 2015 Jun 23; 131(25):2185–93. 
  17. Gussak I, Brugada P, Brugada J, Wright RS, Kopecky SL, Chaitman BR, et al. Idiopathic short QT interval: a new clinical syndrome? Cardiology. 2000; 94(2):99–102. 
  18. Harrell DT, Ashihara T, Ishikawa T, Tominaga I, Mazzanti A, Takahashi K, et al. Genotype-dependent differences in age of manifestation and arrhythmia complications in short QT syndrome. Int J Cardiol. 2015 Jul 1; 190:393–402. 
  19. Mazzanti A, Kanthan A, Monteforte N, Memmi M, Bloise R, Novelli V, et al. Novel insight into the natural history of short QT syndrome. J Am Coll Cardiol. 2014 Apr 8; 63(13):1300–8. 
  20. Gaita F, Giustetto C, Bianchi F, Wolpert C, Schimpf R, Riccardi R, et al. Short QT Syndrome: a familial cause of sudden death. Circulation. 2003 Aug 26; 108(8):965–70. 
  21. Giustetto C, Schimpf R, Mazzanti A, Scrocco C, Maury P, Anttonen O, et al. Long-term follow-up of patients with short QT syndrome. J Am Coll Cardiol. 2011 Aug 2; 58(6):587–95. 
  22. Gaita F, Giustetto C, Bianchi F, Schimpf R, Haissaguerre M, Calò L, et al. Short QT syndrome: pharmacological treatment. J Am Coll Cardiol. 2004 Apr 21; 43(8):1494–9. 

Genetik und klinische Diagnostik der hypertrophen Kardiomyopathie

Hermann Eichstädt, Martin Steeg, Bernadette Eichstädt, Thomas Walkowiak, Felicitas Escher, Wolfgang Bocksch und Burkert Pieske


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  34. Jongbloed RJ, Marcelis CL, Doevendans PA, Schmeitz-Mulkens JM, Van Dockum WG, Geraedts JP, Smeets HJ. Variable clinical manifestation of a novel missense mutation in the alpha-tropomyosin (TPM1) gene in familial hypertrophic cardiomyopathy. J Am Coll Cardiol 2003 Mar 19; 41(6): 981–6.
  35. Kokado H, Shimizu M, Yoshio H, Ino H, Okeie K, Emoto Y, Matsuyama T, Yamaguchi M, Yasuda T, Fujino N, Ito H, Mabuchi H. Clinical features of hypertrophic cardiomyopathy caused by a Lys183 deletion mutation in the cardiac troponin I gene. Circulation 2000 Aug 8; 102(6): 663–9.
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Medikamentöse Therapieansätze bei hypertropher und dilatativer Kardiomyopathie

Hermann Eichstädt, Martin Steeg, Bernadette Eichstädt, Thomas Walkowiak, Wolfgang Bocksch und Burkert Pieske


  1. Elliott, P. M. and the Task Force members: 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),  European Heart Journal 2014; 35: 2733–2779.
  2. Frey, N., Pieske, B., Steven, D., Tillmanns, C., Baldus, S. Kommentar zu den 2014 ESC Guidelines zur Diagnostik und Therapie der hypertrophen Kardiomyopathie. Kardiologe 2016; 10: 24–27.
  3. Frey, N., Pieske, B., Steven, D., Tillmanns, C. Pocket-Leitlinie: Diagnose und Behandlung der hypertrophen Kardiomyopathie. Börm Bruckmeier Verlag GmbH, Grünwald, 2016.
  4. Eichstädt, H., Störk, T., Sommerfeld, J., Sommerfeld, R., Walkowiak, T. Gutberlet, M. Kardiovaskuläre MRT – Update. Med Welt 2007; 58: 465–471.
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Hypertrophe obstruktive Kardiomyopathie – weitere therapeutische Optionen

David Sinning, Ulf Landmesser


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