Journal Mobile Options
Table of Contents
Vol. 16, No. 6, 2001
Issue release date: November–December 2001
Fetal Diagn Ther 2001;16:433–436

Prenatal Echocardiographic Appearance of Arrhythmogenic Right Ventricle Dysplasia: A Case Report

Rustico M.A. · Benettoni A. · Fontaliran F. · Fontaine G.
Departments of aObstetrics and Gynecology and bCardiology, Istituto per l’Infanzia, Trieste, Italy; cService d’Anatomie-Pathologique, Hôpital Boucicaut, Paris, and dDepartment of Cardiology, Hôpital Jean Rostand, Ivry sur Seine, France

Individual Users: Register with Karger Login Information

Please create your User ID & Password

Contact Information

I have read the Karger Terms and Conditions and agree.

To view the fulltext, please log in

To view the pdf, please log in


We report a case of arrhythmogenic right ventricular dysplasia (ARVD) diagnosed prenatally by echocardiography at 24 weeks gestation. The 4-chamber view showed a large outpouched area extending from below the tricuspid valve to the insertion of the moderator band; the affected wall appeared thin and akinetic, with absence of flow at color Doppler investigation and no evidence of cardiovascular failure. The size of the outpouched area was unchanged at subsequent controls (25 and 26 weeks gestation) when frequent extrasystoles occurred, probably of a ventricular origin. The pregnancy was terminated at 27 weeks. The histopathologic examination of the fetal heart showed the presence of clusters of adipocytes interspersed with myocardial fibers, consistent with the diagnosis of ARVD.

Copyright / Drug Dosage

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.


  1. Thiene G, Nava A, Corrado D, Rossi L, Pennelli N: Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med 1988;318:129–133.
  2. Fontaine G, Fontaliran F, Zenati O, Guzman CE, Rigoulet J, Berthier J, Frank R: Fat in the heart. A feature unique to the human species? Observational reflections on an unsolved problem. Acta Cardiol 1999;54:189–194.
  3. Fontaine G, Fontaliran F, Frank R: Arrhythmogenic right ventricular cardiomyopathies. Clinical forms and main differential diagnoses. Circulation 1998;97:1532–1535.
  4. Fontaliran F, Arkwright S, Vildè F, Fontaine G: Dysplasie et cardiomyopathie ventriculaire droite arythmogènes. Arch Anat Cytol Pathol 1998;46:171–177.
  5. Sing S, Katkov H, Zavoral JH, Sane S, Mc Leod JD: Congenital aneurysms of the left ventricle. Am Heart J 1980;99:25–32.
  6. Sepulveda W, Drysdale K, Kyle P, McNeal AD, Moore IE: Congenital left ventricular aneurysm causing hydrops fetalis: Prenatal diagnosis with color Doppler ultrasonography. J Ultrasound Med 1996;15:327–331.
  7. Jacobson RL, Perez A, Meyer RA, Miodovnik M, Siddiqi TA: Prenatal diagnosis of fetal left ventricular aneurysm: A case report and a review. Obstet Gynecol 1991;78:525–528.

    External Resources

  8. Matias A, Fredouille C, Nesmann C, Azancot A: Prenatal diagnosis of left ventricular aneurysm: A report of three cases and a review. Cardiol Young 1999;9:175–184.
  9. Hornberger LK, Dalvi B, Benacerraf BR: Prenatal sonographic detection of cardiac aneurysms and diverticula. J Ultrasound Med 1994;13:967–970.
  10. Cavallè-Garrido T, Cloutier A, Harder J, Boutin C, Smallhorn JF: Evolution of fetal ventricular aneurysms and diverticula of the heart: An echocardiographic study. Am J Perinat 1997;14:393–400.
  11. Gerlis LM, Schmidt-Ott SC, Ho SY, Anderson RH: Dysplastic condition of the right ventricular myocardium: Uhl’s anomaly v arrhythmogenic right ventricular dysplasia. Br Heart J 1993;69:142–150.
  12. Wager GP, Couser RJ, Edwards OP, Gmach C, Bessinger B: Antenatal ultrasound findings in a case of Uhl’s anomaly. Am J Perinat 1988;5:164–167.

    External Resources

  13. Benson CB, Brown DL, Roberts D: Uhl’s anomaly of the heart mimicking Ebstein’s anomaly in utero. J Ultrasound Med 1995;14:781–783.
  14. Pinamonti B, Sinagra G, Camerini F: Clinical relevance of right ventricular dysplasia/cardiomyopathy. Heart 2000;83:9–11.
  15. Pawel BR, de-Chadarevian JP, Wolk JH, Donner RM, Vogel RL, Braverman P: Sudden death in childhood due to right ventricular dysplasia: Report of two cases. Pediatr Pathol 1994;14:987–995.
  16. James TN, Nichols MM, Sapire DW, Di Patre PL, Lopez S: Complete heart block and fatal right ventricular failure in an infant. Circulation 1996;93:1588–1600.
  17. Corrado D, Basso C, Thiene G, McKenna W, Davies MJ, Fontaliran F, Nava A, Silvestri F, Blomstrom-Lundqvist C, Wlodarska E, Fontaine G, Camerini F: Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: A multicenter study. JACC 1997;30:1512–1520.
  18. McKenna WJ, Thiene G, Nava A, Fontaliran F, Blomstrom-Lundqvist C, Fontaine G, Camerini F: Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Br Heart J 1994;71:215–218.
  19. Case C, Wiles H, Gillette P, Oslizlok P: Fetal and neonatal dysrhythmia associated with a ventricular aneurysm. Am Heart J 1989;118:849–851.

    External Resources

  20. Pinamonti B, Miani D, Sinagra G, Bussani R, Silvestri F, Camerini F: Familial right ventricular dysplasia with biventricular involvement and inflammatory infiltration. Heart 1996;76:66–69.

Pay-per-View Options
Direct payment This item at the regular price: USD 33.00
Payment from account With a Karger Pay-per-View account (down payment USD 150) you profit from a special rate for this and other single items.
This item at the discounted price: USD 23.00