Pediatric Cardiology

, Volume 11, Issue 2, pp 113–116 | Cite as

Arrhythmogenic right ventricular dysplasia in brother and sister: Is it related to myocarditis?

  • Karl-Göran Sabel
  • Carina Blomström-Lundqvist
  • S. Bertil Olsson
  • Sverker Eneström
Case Reports


Two cases of arrhythmogenic right ventricular dysplasia (ARVD) in siblings are reported. In the boy, 14 years old, the clinical history, ECG, echocardiography, and histopathological findings were consistent with ARVD. Premature ventricular contractions of left bundle branch block (LBBB) pattern were recorded but no ventricular tachycardia (VT). A high titer against mycoplasma and increased concentrations of immunoglobulins were found. Two years after his first admission he died suddenly. Autopsy revealed severe right ventricular (RV) myocardial damage, with fat cell infiltration and collagenous tissue. His sister presented with sustained VT of LBBB pattern 2 years later, at 12 years of age. Vaccination against rubella and signs of upper respiratory illness had preceded the symptoms. During the following 9 days ECGs and serum enzymes indicated the development of left ventricular (LV) infarction. Echocardiography revealed an enlarged RV and a normal LV. After 6 weeks both RV and LV showed akinetic areas and sacculations. We suggest that myocarditis may be a precipitating factor in ARVD, and perhaps the prerequisite for its manifestation.

Key Words

Arrhythmogenic right ventricular dysplasia Dilated cardiomyopathy Myocarditis Familial occurrence 


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  1. 1.
    Blomström-Lundqvis C, Sabel K-G, Olsson SB (1988) A long-term follow-up of 15 patients with arrhythmogenic right ventricular dysplasia.Br Heart J 58:477–488Google Scholar
  2. 2.
    Costanzo-Nordin MR, O'Connel JB, Subramanian R, Robinson JA, Scanlon RJ (1985) Myocarditis confirmed by biopsy presenting as acute myocardial infarction.Br Heart J 53:25–29Google Scholar
  3. 3.
    Dec W Jr, Palacios IF, Fallon JT, Aretz HT, Mills J, Lee DC-S, Johnson RA (1985) Active myocarditis in the spectrum of acute dilated cardiomyopathies.N Engl J Med 312:885–890Google Scholar
  4. 4.
    Dungan WT, Garson A Jr, Gilette PC (1981) Arrhythmogenic right ventricular dysplasia: A cause of ventricular tachycardia in children with apparently normal hearts.Am Heart J 102:745–750Google Scholar
  5. 5.
    Fitchett DH, Sugrue DD, MacArthur CG, Oakley CM (1984) Right ventricular dilated cardiomyopathy.Br Heart J 51:25–29Google Scholar
  6. 6.
    Fontaine G, Guiraudon G, Frank R, Tereau Y, Fillette F, Marcus FI, Chomette G, Grosgogeat Y (1982) Dysplasie ventriculaire arythmogene et maladie de Uhl.Arch Mal Caeur 75:361–371Google Scholar
  7. 7.
    Ibsen HHW, Baandrup U, Simonsen EE (1985) Familial right ventricular dilated cardiomyopathy.Br Heart J 54:156–159Google Scholar
  8. 8.
    Manyari DE, Klein GJ, Gulamhusein S, Boughner D, Guiraudon GM, Wyse G, Mitchell B, Kostuk WJ (1983) Arrhythmogenic right ventricular dysplasia: A generalized cardiomyopathy?Circulation 68:251–257Google Scholar
  9. 9.
    Marcus FI, Fontaine GA, Guiraudon G, Frank R, Laurenceau JL, Malergue C, Grosgogeat Y (1982) Right ventricular dysplasia: A report of 24 adult cases.Circulation 65:384–398Google Scholar
  10. 10.
    Matsumori A, Kawai C (1980) Coxsackie virus B3 perimyocarditis in balb/c mice: Experimental model of chronic perimyocarditis in the right ventricle.J Pathol 131:97–106Google Scholar
  11. 11.
    Nava A, Scognamiglio R, Thiene G, Canciani B, Daliento L, Buja G, Stritoni P, Fasoli G, Dalla Volta S (1987) A polymorphic form of familial arrhythmogenic right ventricular dysplasia.Am J Cardiol 59:1405–1409Google Scholar
  12. 12.
    Olsson SB, Edvardsson N, Emanuelsson H, Eneström S (1982) A case of arrhythmogenic right ventricular dysplasia with ventricular fibrillation.Clin Cardiol 5:591–596Google Scholar
  13. 13.
    Pearce JM (1960) Heart disease and filtrable viruses.Circulation 21:448–455Google Scholar
  14. 14.
    Pönkä A (1979) Carditis associated withMycoplasma pneumoniae infection.Acta Med Scand 206:77–86Google Scholar
  15. 15.
    Rakovec P, Rossi L, Fontaine G, Sasel B, Markez J, Voncina D (1986) Familial arrhythmogenic right ventricular disease.Am J Cardiol 58:377–378Google Scholar
  16. 16.
    Saffitz JE, Schwartz DJ, Southworth W, Murphree S, Rodriguez ER, Ferrans VJ, Roberts WC (1982) Coxsackie viral myocarditis causing transmural right and left ventricular infarction without coronary narrowing.Am J Cardiol 52:644–647Google Scholar

Copyright information

© Springer-Verlag New York Inc. 1990

Authors and Affiliations

  • Karl-Göran Sabel
    • 1
  • Carina Blomström-Lundqvist
    • 2
  • S. Bertil Olsson
    • 2
  • Sverker Eneström
    • 3
  1. 1.Section of Paediatric Cardiology, Department of PaediatricsEast HospitalGöteborg
  2. 2.Division of Cardiology, Med Department ISahlgrenska HospitalGöteborg
  3. 3.Department of PathologyUniversity HospitalLinköpingSweden

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