Case Report: Nonarrhythmogenic Right Ventricular Dysplasia Presenting with Severe Right Ventricular Failure in an Adolescent


  • Mustafa Pac
  • Aysenur Pac
  • Tugcin Bora Polat
  • Sevket Balli
  • Nesrin Turhan
  • Dursun Aras
  • Mehmet Burhan Oflaz
  • Seref Kücüker



Right ventricular dysplasia is usually discovered by the presence of ventricular arrhythmia. As arrhythmia is an epiphenomenon, the first presentation of some cases can be primarily heart failure. We describe an adolescent girl who presented with progressive right heart failure and whose hallmark was fibrofatty replacement of ventricular muscle, especially of the right side, without ventricular arrhythmia. The patient was successfully treated by orthotopic heart transplantation.


Blasco PB, Comas JG. 2006. Severe arrhythmogenic right ventricular dysplasia without fatty infiltration on magnetic resonance imaging: fibrofatty form. Pediatr Cardiol 27:476-7.nBurke AP, Farb A, Tashko G, et al. 1998. Arrhythmogenic right ventricular cardiomyopathy and fatty replacement of the right ventricular myocardium: Are they different diseases? Circulation 97:1571-80.nCorrado D, Basso C, Thiene G. 2000. Arrhythmogenic right ventricular cardiomyopathy: diagnosis, prognosis, and treatment. Heart 83:588-95.nCubero JM, Gallego P, Pavon M. 2002. Arrhythmogenic right ventricular cardiomyopathy and atrial right-to-left shunt. Acta Cardiol 57:443-5.nDalal D, Nasir K, Bomma C, et al. 2005. Arrhythmogenic right ventricular dysplasia. A United States experience. Circulation 112:3823-32.nFontaine G, Fontaliran F, Frank R. 1998. Arrhythmogenic right ventricular cardiomyopathies: clinical forms and main differential diagnoses. Circulation 97:1532-5.nFontaliran F, Fontaine G, Fillette F, et al. 1991. Frontières nosologiques de la dysplasie arythmogene: variations quantitatives du tissu adipeux ventriculaire droit normal. Arch Mal Coeur 84:33-8.nGilljam T, Bergh CH. 2009. Right ventricular cardiomyopathy: timing of heart transplantation in Uhl's anomaly and arrythmogenic right ventricular cardiomyopathy. Eur J Heart Fail 11:106-9.nGirard F, Fontaine G, Fontaliran F, et al. 1997. Catastrophic global heart failure in a patient with non-arrhythmogenic right ventricular dysplasia. Heart Vessels 12:152-4.nGorgulu S, Nurkalem Z, Celebi A, et al. 2006. Unusual presentation of a patient with arrhythmogenic right ventricular dysplasia treated with a Glenn shunt. Int J Cardiol 113:410-3.nHoffman BF, Feinmark SJ, Guo SD. 1997. Electrophysiologic effects of interactions between activated canine neutrophils and cardiac myocytes. J Cardiovasc Electrophysiol 8:679-87.nMarcus FI, Fontaine GH, Guiraudon G, et al. 1982. Right ventricular dysplasia: a report of 24 adult cases. Circulation 65:384-98.nMorita K, Takeuchi M, Oe K, et al. 2002. Perioperative management of Fontan operation for two patients with arrhythmogenic right ventricular dysplasia. J Anesth 16:169-72.nPinamonti B, Sinagra G, Salvi A, et al. 1992. Left ventricular involvement in right ventricular dysplasia. Am Heart J 123:711-24.nRichardson P, McKenna W, Bristow M, et al. 1996. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of Cardiomyopathies. Circulation 93:841-2.nTandri H, Calkins H, Nasir K, et al. 2003. Magnetic resonance imaging findings in patients meeting task force criteria for arrhythmogenic right ventricular dysplasia. J Cardiovasc Electrophysiol 14:476-82.nWebb JG, Kerr CR, Huckell VF, et al. 1986. Right ventricular abnormalities in arrhythmogenic right ventricular dysplasia. Am J Cardiol 58:568-70.n



How to Cite

Pac, M., Pac, A., Polat, T. B., Balli, S., Turhan, N., Aras, D., Oflaz, M. B., & Kücüker, S. (2010). Case Report: Nonarrhythmogenic Right Ventricular Dysplasia Presenting with Severe Right Ventricular Failure in an Adolescent. The Heart Surgery Forum, 13(1), E21-E24.




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