Skip to main content
NCBI home page
Heart logoLink to Heart
. 1996 Jul;76(1):66–69. doi: 10.1136/hrt.76.1.66

Familial right ventricular dysplasia with biventricular involvement and inflammatory infiltration. Heart Muscle Disease Study Group.

B Pinamonti 1, D Miani 1, G Sinagra 1, R Bussani 1, F Silvestri 1, F Camerini 1
PMCID: PMC484429  PMID: 8774331

Abstract

The aetiology of right ventricular dysplasia/cardiomyopathy is presently unknown. A genetic background has been suggested, but myocarditis may play a part in its pathogenesis. Two familial cases of right ventricular dysplasia, one of whom had also a diagnosis of myocarditis, are reported. Both patients presented with ventricular arrhythmias. The father subsequently had a "flu-like" syndrome, heart failure, and biventricular dysfunction; "active" myocarditis was found at endomyocardial biopsy. Then the patient died suddenly. The daughter developed progressive biventricular dysfunction; then she was resuscitated from a cardiac arrest, and subsequently died suddenly. In both patients necropsy showed severe right ventricular atrophy and fibro-adipose substitution, associated with biventricular fibrosis. Inflammatory infiltration was also present in the first patient. This study shows the association of right ventricular dysplasia and myocarditis in the same family. These cases may represent a link between inherited and acquired ("inflammatory") forms of the disease.

Full text

PDF
66

Images in this article

67Image
on p.67
67Image
on p.67
67Image
on p.67
68Image
on p.68

Selected References

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

  1. Aretz H. T., Billingham M. E., Edwards W. D., Factor S. M., Fallon J. T., Fenoglio J. J., Jr, Olsen E. G., Schoen F. J. Myocarditis. A histopathologic definition and classification. Am J Cardiovasc Pathol. 1987 Jan;1(1):3–14. [PubMed] [Google Scholar]
  2. Bharati S., Feld A. W., Bauernfeind R., Kattus A. A., Lev M. Hypoplasia of the right ventricular myocardium with ventricular tachycardia. Arch Pathol Lab Med. 1983 May;107(5):249–253. [PubMed] [Google Scholar]
  3. Björkhem G., Evander E., White T., Lundström N. R. Myocardial scintigraphy with 201thallium in pediatric cardiology: a review of 52 cases. Pediatr Cardiol. 1990 Jan;11(1):1–7. doi: 10.1007/BF02239540. [DOI] [PubMed] [Google Scholar]
  4. 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]
  5. Hofmann R., Trappe H. J., Klein H., Kemnitz J. Chronic (or healed) myocarditis mimicking arrhythmogenic right ventricular dysplasia. Eur Heart J. 1993 May;14(5):717–720. doi: 10.1093/eurheartj/14.5.717. [DOI] [PubMed] [Google Scholar]
  6. Imakita M., Yutani C., Ishibashi-Ueda H., Isobe F., Kamiya T. A patient with ventricular tachycardia showing remarkable fatty infiltration and lymphocytic myocarditis in the right ventricular wall. Am J Cardiovasc Pathol. 1990;3(4):337–340. [PubMed] [Google Scholar]
  7. 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]
  8. Matsumori A., Kawai C. Coxsackie virus B3 perimyocarditis in BALB/c mice: experimental model of chronic perimyocarditis in the right ventricle. J Pathol. 1980 Jun;131(2):97–106. doi: 10.1002/path.1711310202. [DOI] [PubMed] [Google Scholar]
  9. McKenna W. J., Thiene G., Nava A., Fontaliran F., Blomstrom-Lundqvist C., Fontaine G., Camerini F. Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology. Br Heart J. 1994 Mar;71(3):215–218. doi: 10.1136/hrt.71.3.215. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. 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]
  11. 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]
  12. O'Connell J. B., Fowles R. E., Robinson J. A., Subramanian R., Henkin R. E., Gunnar R. M. Clinical and pathologic findings of myocarditis in two families with dilated cardiomyopathy. Am Heart J. 1984 Jan;107(1):127–135. doi: 10.1016/0002-8703(84)90146-7. [DOI] [PubMed] [Google Scholar]
  13. Pinamonti B., Alberti E., Cigalotto A., Dreas L., Salvi A., Silvestri F., Camerini F. Echocardiographic findings in myocarditis. Am J Cardiol. 1988 Aug 1;62(4):285–291. doi: 10.1016/0002-9149(88)90226-3. [DOI] [PubMed] [Google Scholar]
  14. Pinamonti B., Di Lenarda A., Sinagra G., Silvestri F., Bussani R., Camerini F. Long-term evolution of right ventricular dysplasia-cardiomyopathy. The Heart Muscle Disease Study Group. Am Heart J. 1995 Feb;129(2):412–415. doi: 10.1016/0002-8703(95)90029-2. [DOI] [PubMed] [Google Scholar]
  15. 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]
  16. Rampazzo A., Nava A., Danieli G. A., Buja G., Daliento L., Fasoli G., Scognamiglio R., Corrado D., Thiene G. The gene for arrhythmogenic right ventricular cardiomyopathy maps to chromosome 14q23-q24. Hum Mol Genet. 1994 Jun;3(6):959–962. doi: 10.1093/hmg/3.6.959. [DOI] [PubMed] [Google Scholar]
  17. 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]
  18. Zolezzi F., Filippi E., Rosso R., Capella G., Nava R., Magrini U., Arbustini E. Chronic myocarditis leading to a right ventricular cardiomyopathy. Case report. G Ital Cardiol. 1986 Mar;16(3):273–275. [PubMed] [Google Scholar]

Articles from Heart are provided here courtesy of BMJ Publishing Group

RESOURCES