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British Heart Journal logoLink to British Heart Journal
. 1987 Nov;58(5):477–488. doi: 10.1136/hrt.58.5.477

A long term follow up of 15 patients with arrhythmogenic right ventricular dysplasia.

C Blomström-Lundqvist 1, K G Sabel 1, S B Olsson 1
PMCID: PMC1277344  PMID: 3676037

Abstract

The clinical course in 15 patients with features consistent with arrhythmogenic right ventricular dysplasia is described. At referral seven patients had abnormal physical findings, nine had abnormal electrocardiograms with non-specific right-sided abnormalities, and seven patients had increased heart size or prominent right ventricles on chest x ray. During long term follow up (mean 8.8 years, range 1.5 to 28 years) 11 patients had abnormal physical findings, 11 had electrocardiographic changes, and nine had increased heart size. Recurrent sustained right ventricular tachycardia was the most common arrhythmia (10 patients). Two patients experienced ventricular fibrillation. Seven patients suffered from over 10 episodes of ventricular tachycardia, nine required cardioversions, and 10 patients had associated serious symptoms such as syncope, severe hypotension, or cardiac arrest. Four patients required operation to correct the arrhythmia and three patients developed right heart failure. Two out of three deaths were sudden. These data suggest that in arrhythmogenic right ventricular dysplasia right ventricular abnormalities may be progressive and that the condition may affect the left ventricle. The course of the ventricular arrhythmias was highly variable and could not be predicted in individual patients. The potential for lethal ventricular arrhythmias is evident and warrants intensive diagnostic efforts to identify patients with adverse prognostic features.

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Selected References

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  1. B-Lundqvist C., Eneström S., Edvardsson N., Olsson S. B. Arrhythmogenic right ventricular dysplasia presenting with ventricular tachycardia in a father and son. Clin Cardiol. 1987 Apr;10(4):277–283. doi: 10.1002/clc.4960100415. [DOI] [PubMed] [Google Scholar]
  2. Baran A., Nanda N. C., Falkoff M., Barold S. S., Gallagher J. J. Two-dimensional echocardiographic detection of arrhythmogenic right ventricular dysplasia. Am Heart J. 1982 Jun;103(6):1066–1067. doi: 10.1016/0002-8703(82)90570-1. [DOI] [PubMed] [Google Scholar]
  3. Beerman L. B., Zuberbuhler J. R., Neches W. H., Fischer D. R., Fricker F. J., Mathews R. A., Park S. C., Lenox C. C. Arrhythmogenic right ventricular dysplasia associated with atrioventricular conduction disturbance. Am J Cardiol. 1983 Oct 1;52(7):909–912. doi: 10.1016/0002-9149(83)90441-1. [DOI] [PubMed] [Google Scholar]
  4. Brugada P., Green M., Abdollah H., Wellens H. J. Significance of ventricular arrhythmias initiated by programmed ventricular stimulation: the importance of the type of ventricular arrhythmia induced and the number of premature stimuli required. Circulation. 1984 Jan;69(1):87–92. doi: 10.1161/01.cir.69.1.87. [DOI] [PubMed] [Google Scholar]
  5. Buxton A. E., Waxman H. L., Marchlinski F. E., Simson M. B., Cassidy D., Josephson M. E. Right ventricular tachycardia: clinical and electrophysiologic characteristics. Circulation. 1983 Nov;68(5):917–927. doi: 10.1161/01.cir.68.5.917. [DOI] [PubMed] [Google Scholar]
  6. Deal B. J., Miller S. M., Scagliotti D., Prechel D., Gallastegui J. L., Hariman R. J. Ventricular tachycardia in a young population without overt heart disease. Circulation. 1986 Jun;73(6):1111–1118. doi: 10.1161/01.cir.73.6.1111. [DOI] [PubMed] [Google Scholar]
  7. Dungan W. T., Garson A., Jr, Gillette P. C. Arrhythmogenic right ventricular dysplasia: a cause of ventricular tachycardia in children with apparently normal hearts. Am Heart J. 1981 Oct;102(4):745–750. doi: 10.1016/0002-8703(81)90101-0. [DOI] [PubMed] [Google Scholar]
  8. Foale R. A., Nihoyannopoulos P., Ribeiro P., McKenna W. J., Oakley C. M., Krikler D. M., Rowland E. Right ventricular abnormalities in ventricular tachycardia of right ventricular origin: relation to electrophysiological abnormalities. Br Heart J. 1986 Jul;56(1):45–54. doi: 10.1136/hrt.56.1.45. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Fontaine G., Frank R., Guiraudon G., Pavie A., Tereau Y., Chomette G., Grosgogeat Y. Signification des troubles de conduction intraventriculaires observés dans la dysplasie ventriculaire droite arythmogène. Arch Mal Coeur Vaiss. 1984 Aug;77(8):872–879. [PubMed] [Google Scholar]
  10. Fontaine G., Guiraudon G., Frank R., Fillette F., Cabrol C., Grosgogeat Y. Surgical management of ventricular tachycardia unrelated to myocardial ischemia or infarction. Am J Cardiol. 1982 Feb 1;49(2):397–410. doi: 10.1016/0002-9149(82)90517-3. [DOI] [PubMed] [Google Scholar]
  11. Fontaine G., Guiraudon G., Frank R., Tereau Y., Fillette F., Marcus F. I., Chomette G., Grosgogeat Y. Dysplasie ventriculaire droite arythmogène et maladie de Uhl. Arch Mal Coeur Vaiss. 1982 Apr;75(4):361–371. [PubMed] [Google Scholar]
  12. Fulton D. R., Chung K. J., Tabakin B. S., Keane J. F. Ventricular tachycardia in children without heart disease. Am J Cardiol. 1985 May 1;55(11):1328–1331. doi: 10.1016/0002-9149(85)90498-9. [DOI] [PubMed] [Google Scholar]
  13. Guiraudon G., Fontaine G., Frank R., Leandri R., Barra J., Cabrol C. Surgical treatment of ventricular tachycardia guided by ventricular mapping in 23 patients without coronary artery disease. Ann Thorac Surg. 1981 Nov;32(5):439–450. doi: 10.1016/s0003-4975(10)61775-4. [DOI] [PubMed] [Google Scholar]
  14. Higuchi S., Cağlar N. M., Shimada R., Yamada A., Takeshita A., Nakamura M. 16-year follow-up of arrhythmogenic right ventricular dysplasia. Am Heart J. 1984 Nov;108(5):1363–1365. doi: 10.1016/0002-8703(84)90769-5. [DOI] [PubMed] [Google Scholar]
  15. Leitl G. P., Buchanan J. W., Wagner H. N., Jr Monitoring cardiac function with nuclear techniques. Am J Cardiol. 1980 Dec 18;46(7):1125–1132. doi: 10.1016/0002-9149(80)90282-9. [DOI] [PubMed] [Google Scholar]
  16. Manyari D. E., Duff H. J., Kostuk W. J., Belenkie I., Klein G. J., Wyse D. G., Mitchell L. B., Boughner D., Guiraudon G., Smith E. R. Usefulness of noninvasive studies for diagnosis of right ventricular dysplasia. Am J Cardiol. 1986 May 1;57(13):1147–1153. doi: 10.1016/0002-9149(86)90690-9. [DOI] [PubMed] [Google Scholar]
  17. Manyari D. E., Klein G. J., Gulamhusein S., Boughner D., Guiraudon G. M., Wyse G., Mitchell L. B., Kostuk W. J. Arrhythmogenic right ventricular dysplasia: a generalized cardiomyopathy? Circulation. 1983 Aug;68(2):251–257. doi: 10.1161/01.cir.68.2.251. [DOI] [PubMed] [Google Scholar]
  18. Marcus F. I., Fontaine G. H., Guiraudon G., Frank R., Laurenceau J. L., Malergue C., Grosgogeat Y. Right ventricular dysplasia: a report of 24 adult cases. Circulation. 1982 Feb;65(2):384–398. doi: 10.1161/01.cir.65.2.384. [DOI] [PubMed] [Google Scholar]
  19. Morgera T., Salvi A., Alberti E., Silvestri F., Camerini F. Morphological findings in apparently idiopathic ventricular tachycardia. An echocardiographic haemodynamic and histologic study. Eur Heart J. 1985 Apr;6(4):323–334. doi: 10.1093/oxfordjournals.eurheartj.a061859. [DOI] [PubMed] [Google Scholar]
  20. Olsson S. B., Edvardsson N., Emanuelsson H., Eneström S. A case of arrhythmogenic right ventricular dysplasia with ventricular fibrillation. Clin Cardiol. 1982 Nov;5(11):591–596. doi: 10.1002/clc.4960051104. [DOI] [PubMed] [Google Scholar]
  21. Panidis I. P., Greenspan A. M., Mintz G. S., Ross J. Inducible ventricular fibrillation in arrhythmogenic right ventricular dysplasia. Am Heart J. 1985 Nov;110(5):1067–1069. doi: 10.1016/0002-8703(85)90213-3. [DOI] [PubMed] [Google Scholar]
  22. Rakovec P., Rossi L., Fontaine G., Sasel B., Markez J., Voncina D. Familial arrhythmogenic right ventricular disease. Am J Cardiol. 1986 Aug 1;58(3):377–378. doi: 10.1016/0002-9149(86)90089-5. [DOI] [PubMed] [Google Scholar]
  23. Reiter M. J., Smith W. M., Gallagher J. J. Clinical spectrum of ventricular tachycardia with left bundle branch morphology. Am J Cardiol. 1983 Jan 1;51(1):113–121. doi: 10.1016/s0002-9149(83)80021-6. [DOI] [PubMed] [Google Scholar]
  24. Ruder M. A., Winston S. A., Davis J. C., Abbott J. A., Eldar M., Scheinman M. M. Arrhythmogenic right ventricular dysplasia in a family. Am J Cardiol. 1985 Nov 1;56(12):799–800. doi: 10.1016/0002-9149(85)91144-0. [DOI] [PubMed] [Google Scholar]
  25. Sahn D. J., DeMaria A., Kisslo J., Weyman A. Recommendations regarding quantitation in M-mode echocardiography: results of a survey of echocardiographic measurements. Circulation. 1978 Dec;58(6):1072–1083. doi: 10.1161/01.cir.58.6.1072. [DOI] [PubMed] [Google Scholar]
  26. Sevick R. J., Wyse D. G. Long-term management of arrhythmogenic right ventricular dysplasia. Can Med Assoc J. 1983 Feb 15;128(4):418–420. [PMC free article] [PubMed] [Google Scholar]
  27. Strain J. E., Grose R. M., Factor S. M., Fisher J. D. Results of endomyocardial biopsy in patients with spontaneous ventricular tachycardia but without apparent structural heart disease. Circulation. 1983 Dec;68(6):1171–1181. doi: 10.1161/01.cir.68.6.1171. [DOI] [PubMed] [Google Scholar]
  28. Webb J. G., Kerr C. R., Huckell V. F., Mizgala H. F., Ricci D. R. Left ventricular abnormalities in arrhythmogenic right ventricular dysplasia. Am J Cardiol. 1986 Sep 1;58(6):568–570. doi: 10.1016/0002-9149(86)90043-3. [DOI] [PubMed] [Google Scholar]
  29. Wellens H. J., Brugada P., Stevenson W. G. Programmed electrical stimulation of the heart in patients with life-threatening ventricular arrhythmias: what is the significance of induced arrhythmias and what is the correct stimulation protocol? Circulation. 1985 Jul;72(1):1–7. doi: 10.1161/01.cir.72.1.1. [DOI] [PubMed] [Google Scholar]

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