Skip to main content

Abstract

Congenital abnormalities of the right ventricular (RV) myocardium are rare and include arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) and Uhl’s anomaly. Though rare, ARVC/D frequently appears in the differential diagnosis of patients with ventricular arrhythmia. We discuss magnetic resonance (MR) and computed tomography (CT) imaging techniques and various aspects of congenital abnormalities of the RV myocardium with special focus on ARVC/D.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 149.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 199.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 279.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Kramer CM, Barkhausen J, Flamm SD, Kim RJ, Nagel E. Standardized cardiovascular magnetic resonance imaging (CMR) protocols. Society for Cardiovascular Magnetic Resonance: Board of Trustees Task Force on Standardized Protocols. J Cardiovasc Magn Reson. 2008;10:35.

    Article  PubMed  Google Scholar 

  2. Fritz J, Solaiyappan M, Tandri H, et al. Right ventricle shape and contraction patterns and relation to magnetic resonance imaging findings. J Comput Assist Tomogr. 2005;29:725–33.

    Article  PubMed  Google Scholar 

  3. Clarke CJ, Gurka MJ, Norton PT, Kramer CM, Hoyer AW. Assessment of the accuracy and reproducibility of RV volume measurements by CMR in congenital heart disease. JACC Cardiovasc Imaging. 2012;5:28–37.

    Article  PubMed  Google Scholar 

  4. Kimura F, Matsuo Y, Nakajima T, et al. Myocardial fat at cardiac imaging: how can we differentiate pathologic from physiologic fatty infiltration? Radiographics. 2010;30:1587–602.

    Article  PubMed  Google Scholar 

  5. Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation. 2010;121:1533–41.

    Article  PubMed  Google Scholar 

  6. Sen-Chowdhry S, Prasad SK, Syrris P, et al. Cardiovascular magnetic resonance in arrhythmogenic right ventricular cardiomyopathy revisited: comparison with task force criteria and genotype. J Am Coll Cardiol. 2006;48:2132–40.

    Article  PubMed  Google Scholar 

  7. Sen-Chowdhry S, Syrris P, McKenna WJ. Role of genetic analysis in the management of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Coll Cardiol. 2007;50:1813–21.

    Article  PubMed  CAS  Google Scholar 

  8. Sen-Chowdhry S, Syrris P, Ward D, et al. Clinical and genetic characterization of families with arrhythmogenic right ventricular dysplasia/cardiomyopathy provides novel insights into patterns of disease expression. Circulation. 2007;115:1710–20.

    Article  PubMed  Google Scholar 

  9. Sen-Chowdhry S, Syrris P, Prasad SK, et al. Left-dominant arrhythmogenic cardiomyopathy: an under-recognized clinical entity. J Am Coll Cardiol. 2008;52:2175–87.

    Article  PubMed  Google Scholar 

  10. Tandri H, Bomma C, Calkins H, Bluemke DA. Magnetic resonance and computed tomography imaging of arrhythmogenic right ventricular dysplasia. J Magn Reson Imaging. 2004;19:848–58.

    Article  PubMed  Google Scholar 

  11. Nava A, Bauce B, Faliento L. Incidence and natural history. In: Nava A, Rossi L, Thiene G, editors. Arrhythmogenic right ventricular cardiomyopathy/dysplasia. Amsterdam: Elsevier; 1997. p. 17–23.

    Google Scholar 

  12. Marcus FI, Fontaine GH, Guiraudon G, et al. Right ventricular dysplasia: a report of 24 adult cases. Circulation. 1982;65:384–98.

    Article  PubMed  CAS  Google Scholar 

  13. Basso C, Thiene G. Adipositas cordis, fatty infiltration of the right ventricle, and arrhythmogenic right ventricular cardiomyopathy. Just a matter of fat? Cardiovasc Pathol. 2005;14:37–41.

    Article  PubMed  Google Scholar 

  14. Basso C, Corrado D, Rossi L, Thiene G. Morbid anatomy. In: Nava A, Rossi L, Thiene G, editors. Arrhythmogenic right ventricular cardiomyopathy/dysplasia. Amsterdam: Elsevier; 1997. p. 71–86.

    Google Scholar 

  15. Corrado D, Basso C, Thiene G, et al. Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. J Am Coll Cardiol. 1997;30:1512–20.

    Article  PubMed  CAS  Google Scholar 

  16. Tandri H, Saranathan M, Rodriguez ER, et al. Noninvasive detection of myocardial fibrosis in arrhythmogenic right ventricular cardiomyopathy using delayed-enhancement magnetic resonance imaging. J Am Coll Cardiol. 2005;45:98–103.

    Article  PubMed  Google Scholar 

  17. Kimura F, Nakajima T. Arrhythmogenic right ventricular cardiomyopathy/dysplasia. In: Kimura F, editor. Clinical cardiac computed tomography 12: cardiomyopathy evaluated by MDCT. Tokyo: Bunkodo Co., Ltd.; 2012. p. 47–52.

    Google Scholar 

  18. Taylor AJ, Cerqueira M, Hodgson JM, et al. ACCF/SCCT/ACR/AHA/ASE/ASNC/NASCI/SCAI/SCMR 2010 appropriate use criteria for cardiac computed tomography. A report of the American College of Cardiology Foundation Appropriate Use Criteria Task Force, the Society of Cardiovascular Computed Tomography, the American College of Radiology, the American Heart Association, the American Society of Echocardiography, the American Society of Nuclear Cardiology, the North American Society for Cardiovascular Imaging, the Society for Cardiovascular Angiography and Interventions, and the Society for Cardiovascular Magnetic Resonance. J Am Coll Cardiol. 2010;56:1864–94.

    Article  PubMed  Google Scholar 

  19. Hamada S, Takamiya M, Ohe T, Ueda H. Arrhythmogenic right ventricular dysplasia: evaluation with electron-beam CT. Radiology. 1993;187:723–7.

    PubMed  CAS  Google Scholar 

  20. Tandri H, Bluemke DA, Ferrari VA, et al. Findings on magnetic resonance imaging of idiopathic right ventricular outflow tachycardia. Am J Cardiol. 2004;94:1441–5.

    Article  PubMed  Google Scholar 

  21. O’Donnell D, Cox D, Bourke J, Mitchell L, Furniss S. Clinical and electrophysiological differences between patients with arrhythmogenic right ventricular dysplasia and right ventricular outflow tract tachycardia. Eur Heart J. 2003;24:801–10.

    Article  PubMed  Google Scholar 

  22. Mohsen A, Panday M, Wetherold S, Jimenez A. Cardiac sarcoidosis mimicking arrhythmogenic right ventricular dysplasia with high defibrillation threshold requiring subcutaneous shocking coil implantation. Heart Lung Circ. 2012;21:46–9.

    Article  PubMed  Google Scholar 

  23. Lorin de la Grandmaison G, Le Bihan C, Durigon M. Assessment of right ventricular lipomatosis by histomorphometry in control adult autopsy cases. Int J Legal Med. 2001;115:105–8.

    Article  PubMed  CAS  Google Scholar 

  24. Rowin EJ, Yucel KE, Salomon R, Maron MS. Adipositas cordis: an uncommon cardiomyopathy identified by cardiovascular magnetic resonance. Circulation. 2010;122:2212–4.

    Article  PubMed  Google Scholar 

  25. Gerlis L. Uhl’s anomaly. Orphanet encyclopedia. 2003. http://www.orpha.net/data/patho/GB/uk-uhl.pdf.

  26. Greer ML, MacDonald C, Adatia I. MRI of Uhl’s anomaly. Circulation. 2000;101:e230–2.

    Article  PubMed  CAS  Google Scholar 

  27. Otmani A, Leborgne L, Renard C, et al. Electrocardiogram, echocardiography, and magnetic resonance imaging characteristics in Uhl’s disease. Circulation. 2007;115:e11–2.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Fumiko Kimura MD .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer Science+Business Media New York

About this chapter

Cite this chapter

Kimura, F., Hagiwara, N. (2014). The Right Myocardium. In: Saremi, F. (eds) Cardiac CT and MR for Adult Congenital Heart Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8875-0_17

Download citation

  • DOI: https://doi.org/10.1007/978-1-4614-8875-0_17

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-8874-3

  • Online ISBN: 978-1-4614-8875-0

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics