Skip to main content
PMC home page
British Heart Journal logoLink to British Heart Journal
. 1993 Feb;69(2):151–157. doi: 10.1136/hrt.69.2.151

Right ventricular dysplasia: a clinical and pathological study of two families with left ventricular involvement.

D Miani 1, B Pinamonti 1, R Bussani 1, F Silvestri 1, G Sinagra 1, F Camerini 1
PMCID: PMC1024942  PMID: 8435241

Abstract

BACKGROUND--Right ventricular dysplasia is a heart muscle disease of unknown cause that is often familial and is anatomically characterised by adipose or fibroadipose infiltration of the right ventricular myocardium. It is generally regarded as a selective disorder of the right ventricle. AIM--To investigate the prevalence and characteristics of left ventricular involvement in two families in which at least one member had right ventricular dysplasia confirmed at necropsy. METHODS AND RESULTS--Eight patients were found to be affected by right ventricular dysplasia. In three of them this was confirmed at necropsy. Echocardiography or angiography or both showed left ventricular involvement in seven. This ranged from localised wall motion abnormalities to moderate or severe left ventricular dysfunction. The disease was progressive in four cases. At necropsy the left ventricular myocardium showed predominant fibrosis and degenerative changes of the myocardial cells. There were areas of myocardial thinning with fatty infiltration at the apex in two patients. CONCLUSIONS--Familial right ventricular dysplasia can be a progressive disorder that affects the left ventricle. Advanced disease may be clinically confused with dilated cardiomyopathy.

Full text

PDF

Images in this article

152Image
on p.152
153Image
on p.153
153Image
on p.153
154Image
on p.154
156Image
on p.156

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Bharati S., Ciraulo D. A., Bilitch M., Rosen K. M., Lev M. Inexcitable right ventricle and bilateral bundle branch block in Uhl's disease. Circulation. 1978 Mar;57(3):636–644. doi: 10.1161/01.cir.57.3.636. [DOI] [PubMed] [Google Scholar]
  2. Blomström-Lundqvist C., Sabel K. G., Olsson S. B. A long term follow up of 15 patients with arrhythmogenic right ventricular dysplasia. Br Heart J. 1987 Nov;58(5):477–488. doi: 10.1136/hrt.58.5.477. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Blomström-Lundqvist C., Selin K., Jonsson R., Johansson S. R., Schlossman D., Olsson S. B. Cardioangiographic findings in patients with arrhythmogenic right ventricular dysplasia. Br Heart J. 1988 May;59(5):556–563. doi: 10.1136/hrt.59.5.556. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Cherrier F., Floquet J., Cuillière M., Neimann J. L. Les dysplasies ventriculaires droites: a propos de 7 observations. Arch Mal Coeur Vaiss. 1979 Jul;72(7):766–773. [PubMed] [Google Scholar]
  5. Daubert C., Descaves C., Foulgoc J. L., Bourdonnec C., Laurent M., Gouffault J. Critical analysis of cineangiographic criteria for diagnosis of arrhythmogenic right ventricular dysplasia. Am Heart J. 1988 Feb;115(2):448–459. doi: 10.1016/0002-8703(88)90494-2. [DOI] [PubMed] [Google Scholar]
  6. Fontaine G., Fontaliran F., Lascault G., Frank R., Tonet J., Chomette G., Grosgogeat Y. Dysplasie transmise et dysplasie acquise. Arch Mal Coeur Vaiss. 1990 Jun;83(7):915–920. [PubMed] [Google Scholar]
  7. Fontaine G., Frank R., Tonet J. L., Guiraudon G., Cabrol C., Chomette G., Grosgogeat Y. Arrhythmogenic right ventricular dysplasia: a clinical model for the study of chronic ventricular tachycardia. Jpn Circ J. 1984 Jun;48(6):515–538. doi: 10.1253/jcj.48.515. [DOI] [PubMed] [Google Scholar]
  8. Halphen C., Beaufils P., Azancot I., Baudouy P., Manne B., Slama R. Tachycardies ventriculaires récidivantes par dysplasie ventriculaire droite. Association à des anomalies du ventricule gauche. Arch Mal Coeur Vaiss. 1981 Sep;74(9):1113–1118. [PubMed] [Google Scholar]
  9. 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]
  10. Ibsen H. H., Baandrup U., Simonsen E. E. Familial right ventricular dilated cardiomyopathy. Br Heart J. 1985 Aug;54(2):156–159. doi: 10.1136/hrt.54.2.156. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Laurent M., Descaves C., Biron Y., Deplace C., Almange C., Daubert J. C. Familial form of arrhythmogenic right ventricular dysplasia. Am Heart J. 1987 Mar;113(3):827–829. doi: 10.1016/0002-8703(87)90728-9. [DOI] [PubMed] [Google Scholar]
  12. Letac B., Tayot J., Barthes P. Infiltration graisseuse du coeur et maladie de Uhl. A propos d'une observatione de lipomatose cardiaque. Arch Mal Coeur Vaiss. 1977 Jan;70(1):107–113. [PubMed] [Google Scholar]
  13. 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]
  14. 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]
  15. Maron B. J. Right ventricular cardiomyopathy: another cause of sudden death in the young. N Engl J Med. 1988 Jan 21;318(3):178–180. doi: 10.1056/NEJM198801213180309. [DOI] [PubMed] [Google Scholar]
  16. Nava A., Canciani B., Thiene G., Scognamiglio R., Buja G., Martini B., Daliento L., Fasoli G., Stritoni P., Dalla Volta S. Analyse du mode de transmission de la dysplasie ventriculaire droite. Arch Mal Coeur Vaiss. 1990 Jun;83(7):923–928. [PubMed] [Google Scholar]
  17. Nava A., Martini B., Thiene G., Buja G. F., Canciani B., Scognamiglio R., Miraglia G., Corrado D., Boffa G. M., Daliento L. La displasia aritmogena del ventricolo destro. Studio su una popolazione selezionata. G Ital Cardiol. 1988 Jan;18(1):2–9. [PubMed] [Google Scholar]
  18. Nava A., Scognamiglio R., Thiene G., Canciani B., Daliento L., Buja G., Stritoni P., Fasoli G., Dalla Volta S. A polymorphic form of familial arrhythmogenic right ventricular dysplasia. Am J Cardiol. 1987 Jun 1;59(15):1405–1409. doi: 10.1016/0002-9149(87)90929-5. [DOI] [PubMed] [Google Scholar]
  19. Nava A., Thiene G., Canciani B., Scognamiglio R., Daliento L., Buja G., Martini B., Stritoni P., Fasoli G. Familial occurrence of right ventricular dysplasia: a study involving nine families. J Am Coll Cardiol. 1988 Nov;12(5):1222–1228. doi: 10.1016/0735-1097(88)92603-4. [DOI] [PubMed] [Google Scholar]
  20. Pinamonti B., Sinagra G., Salvi A., Di Lenarda A., Morgera T., Silvestri F., Bussani R., Camerini F. Left ventricular involvement in right ventricular dysplasia. Am Heart J. 1992 Mar;123(3):711–724. doi: 10.1016/0002-8703(92)90511-s. [DOI] [PubMed] [Google Scholar]
  21. Purcaro A., Capestro F., Ciampani N., Breccia Fratadocchi G., Belardinelli R., Murer B., Schicchi F., Massini C. La displasia ventricolare destra aritmogena. A proposito di tre osservazioni. G Ital Cardiol. 1984 Dec;14(12):1087–1096. [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. 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]
  24. Thiene G., Nava A., Corrado D., Rossi L., Pennelli N. Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med. 1988 Jan 21;318(3):129–133. doi: 10.1056/NEJM198801213180301. [DOI] [PubMed] [Google Scholar]
  25. Waller B. F., Smith E. R., Blackbourne B. D., Arce F. P., Sarkar N. N., Roberts W. C. Congenital hypoplasia of portions of both right and left ventricular myocardial walls. Clinical and necropsy observations in two patients with parchment heart syndrome. Am J Cardiol. 1980 Nov;46(5):885–891. doi: 10.1016/0002-9149(80)90444-0. [DOI] [PubMed] [Google Scholar]
  26. 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]
  27. Yutani C., Imakita M., Ishibashi-Ueda H., Nagata S., Sakakibara H., Nimura Y. Uhl's anomaly as a result of progression to ventricular dilation from hypertrophic cardiomyopathy. Acta Pathol Jpn. 1987 Sep;37(9):1477–1488. doi: 10.1111/j.1440-1827.1987.tb02269.x. [DOI] [PubMed] [Google Scholar]

Articles from British Heart Journal are provided here courtesy of BMJ Publishing Group

RESOURCES