Journal Mobile Options
Table of Contents
Vol. 30, No. 4, 2011
Issue release date: December 2011
Fetal Diagn Ther 2011;30:280–288

Echocardiographic Risk Stratification of Fetuses with Sacrococcygeal Teratoma and Twin-Reversed Arterial Perfusion

Byrne F.A. · Lee H. · Kipps A.K. · Brook M.M. · Moon-Grady A.J.
aDivision of Cardiology, Department of Pediatrics, University of California San Francisco Benioff Children’s Hospital, bDepartment of Surgery, Pediatric Heart Center, cFetal Treatment Center, and dDivision of Pediatric Surgery, Department of Surgery, University of California San Francisco, San Francisco, Calif., USA

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


Objective: To evaluate pre-intervention echocardiographic parameters of cardiac function in fetuses who survive without hydrops as compared to fetuses who develop hydrops or perinatal death in the setting of sacrococcygeal teratoma (SCT) and twin-reversed arterial perfusion sequence (TRAP). Methods: Clinical, echocardiographic and sonographic data of fetuses with SCT or TRAP during 1999–2009 were reviewed retrospectively. Measurements of cardiothoracic ratio (CTR), cardiac dimension Z-scores, combined ventricular output (CVO), valvular regurgitation, and cardiovascular profile scores (CVPS) were obtained. Results: In total, 19 fetuses (11 SCT, 8 TRAP) met the inclusion criteria and 26 detailed fetal echocardiographic studies were reviewed. Outcome was poor in 7 pregnancies (group A) and good in 12 (group B). Group A had worse CVPS (8.5 vs. 10, p < 0.01) and higher CTR (0.37 vs. 0.30, p = 0.04). At least one of the following was present in each group A fetus: CTR >0.5, CVO >550 ml/min/kg, tricuspid or mitral regurgitation, or mitral valve Z-score >2. No group B fetus had any of these abnormalities. No fetus in either group had abnormal venous Doppler waveforms at presentation. Conclusions: Fetal echocardiography can identify abnormalities of cardiac size and systolic, but not diastolic, function in all fetuses who subsequently died or developed hydrops.

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. Hedrick HL, Flake AW, Crombleholme TM, Howell LJ, Johnson MP, Wilson RD, Adzick NS: Sacrococcygeal teratoma: prenatal assessment, fetal intervention, and outcome. J Pediatr Surg 2004;39:430–438.
  2. Sy ED, Filly RA, Cheong ML, Clifton MS, Cortes RA, Ohashi S, Takifuji K, Ventura DE, Grethel EJ, Wagner AJ, Gress EA, Ball RH, Lee H, Harrison MR: Prognostic role of tumor-head volume ratio in fetal sacrococcygeal teratoma. Fetal Diagn Ther 2009;26:75–80.
  3. Neubert S, Trautmann K, Tanner B, Steiner E, Linke F, Bahlmann F: Sonographic prognostic factors in prenatal diagnosis of sct. Fetal Diagn Ther 2004;19:319–326.
  4. Bond SJ, Harrison MR, Schmidt KG, Silverman NH, Flake AW, Slotnick RN, Anderson RL, Warsof SL, Dyson DC: Death due to high-output cardiac failure in fetal sacrococcygeal teratoma. J Pediatr Surg 1990;25:1287–1291.
  5. Flake AW, Harrison MR, Adzick NS, Laberge JM, Warsof SL: Fetal sacrococcygeal teratoma. J Pediatr Surg 1986;21:563–566.
  6. Moore TR, Gale S, Benirschke K: Perinatal outcome of forty-nine pregnancies complicated by acardiac twinning. Am J Obstet Gynecol 1990;163:907–912.
  7. Okada T, Sasaki F, Cho K, Honda S, Naito S, Hirokata G, Todo S: Management and outcome in prenatally diagnosed sacrococcygeal teratomas. Pediatr Int 2008;50:576–580.
  8. Rand L, Lee H: Complicated monochorionic twin pregnancies: updates in fetal diagnosis and treatment. Clin Perinatol 2009;36:417–430, x–xi.
  9. Chisholm CA, Heider AL, Kuller JA, von Allmen D, McMahon MJ, Chescheir NC: Prenatal diagnosis and perinatal management of fetal sacrococcygeal teratoma. Am J Perinatol 1999;16:47–50.
  10. Schmidt KG, Silverman NH, Harison MR, Callen PW: High-output cardiac failure in fetuses with large sacrococcygeal teratoma: diagnosis by echocardiography and doppler ultrasound. J Pediatr 1989;114:1023–1028.
  11. Kinsel-Ziter ML, Cnota JF, Crombleholme TM, Michelfelder EC: Twin-reversed arterial perfusion sequence: pre- and postoperative cardiovascular findings in the ‘pump’ twin. Ultrasound Obstet Gynecol 2009;34:550–555.
  12. Olutoye OO, Johnson MP, Coleman BG, Crombleholme TM, Adzick NS, Flake AW: Abnormal umbilical cord Doppler sonograms may predict impending demise in fetuses with sacrococcygeal teratoma: a report of two cases. Fetal Diagn Ther 2004;19:35–39.
  13. Livingston JC, Lim FY, Polzin W, Mason J, Crombleholme TM: Intrafetal radiofrequency ablation for twin reversed arterial perfusion (TRAP): a single-center experience. Am J Obstet Gynecol 2007;197:399.e1–e3.
  14. Lee H, Wagner AJ, Sy E, Ball R, Feldstein VA, Goldstein RB, Farmer DL: Efficacy of radiofrequency ablation for twin-reversed arterial perfusion sequence. Am J Obstet Gynecol 2007;196:459.e1–e4.
  15. Adzick NS: Open fetal surgery for life-threatening fetal anomalies. Semin Fetal Neonatal Med 2010;15:1–8.
  16. Geirsson RT, Christie AD, Patel N: Ultrasound volume measurements comparing a prolate ellipsoid method with a parallel planimetric area method against a known volume. J Clin Ultrasound 1982;10:329–332.
  17. Schneider C, McCrindle BW, Carvalho JS, Hornberger LK, McCarthy KP, Daubeney PE: Development of z-scores for fetal cardiac dimensions from echocardiography. Ultrasound Obstet Gynecol 2005;26:599–605.
  18. Tei C, Ling LH, Hodge DO, Bailey KR, Oh JK, Rodeheffer RJ, Tajik AJ, Seward JB: New index of combined systolic and diastolic myocardial performance: a simple and reproducible measure of cardiac function – a study in normals and dilated cardiomyopathy. J Cardiol 1995;26:357–366.
  19. Falkensammer CB, Paul J, Huhta JC: Fetal congestive heart failure: correlation of TEI-index and cardiovascular-score. J Perinat Med 2001;29:390–398.
  20. Hofstaetter C, Hansmann M, Eik-Nes SH, Huhta JC, Luther SL: A cardiovascular profile score in the surveillance of fetal hydrops. J Matern Fetal Neonatal Med 2006;19:407–413.
  21. Jelin E, Hirose S, Rand L, Curran P, Feldstein V, Guevara-Gallardo S, Jelin A, Gonzales K, Goldstein R, Lee H: Perinatal outcome of conservative management versus fetal intervention for twin reversed arterial perfusion sequence with a small acardiac twin. Fetal Diagn Ther 2010;27:138–141.
  22. Kleinman CS, Hobbins JC, Jaffe CC, Lynch DC, Talner NS: Echocardiographic studies of the human fetus: prenatal diagnosis of congenital heart disease and cardiac dysrhythmias. Pediatrics 1980;65:1059–1067.
  23. Kovalchin JP, Silverman NH: The impact of fetal echocardiography. Pediatr Cardiol 2004;25:299–306.
  24. McElhinney DB, Tworetzky W, Lock JE: Current status of fetal cardiac intervention. Circulation 2010;121:1256–1263.
  25. Manganaro L, Savelli S, Di Maurizio M, Perrone A, Tesei J, Francioso A, Angeletti M, Coratella F, Irimia D, Fierro F, Ventriglia F, Ballesio L: Potential role of fetal cardiac evaluation with magnetic resonance imaging: preliminary experience. Prenat Diagn 2008;28:148–156.
  26. Guimaraes CV, Kline-Fath BM, Linam LE, Calvo Garcia MA, Rubio EI, Lim FY: MRI findings in multifetal pregnancies complicated by twin reversed arterial perfusion sequence (TRAP). Pediatr Radiol 2011;41:694–701.
  27. Nemec SF, Horcher E, Kasprian G, Brugger PC, Bettelheim D, Amann G, Nemec U, Rotmensch S, Rimoin DL, Graham JM Jr, Prayer D: Tumor disease and associated congenital abnormalities on prenatal MRI. Eur J Radiol 2011, Epub ahead of print.
  28. Adzick NS: Open fetal surgery for life-threatening fetal anomalies. Semin Fetal Neonatal Med 2010;15:1–8.
  29. Roberts D, Gates S, Kilby M, Neilson JP: Interventions for twin-twin transfusion syndrome: a Cochrane review. Ultrasound Obstet Gynecol 2008;31:701–711.
  30. Kohl T, Ziemann M, Weinbach J, Tchatcheva K, Gembruch U, Hasselblatt M: Partial amniotic carbon dioxide insufflation during minimally invasive fetoscopic interventions seems safe for the fetal brain in sheep. J Laparoendosc Adv Surg Tech A 2010;20:651–653.
  31. Brassard M, Fouron JC, Leduc L, Grignon A, Proulx F: Prognostic markers in twin pregnancies with an acardiac fetus. Obstet Gynecol 1999;94:409–414.
  32. Edelstone DI, Rudolph AM: Preferential streaming of ductus venosus blood to the brain and heart in fetal lambs. Am J Physiol 1979;237:H724–H729.
  33. Mielke G, Benda N: Cardiac output and central distribution of blood flow in the human fetus. Circulation 2001;103:1662–1668.
  34. Gaillard E, Kadem L, Clavel MA, Pibarot P, Durand LG: Optimization of doppler echocardiographic velocity measurements using an automatic contour detection method. Ultrasound Med Biol 2010;36:1513–1524.

Pay-per-View Options
Direct payment This item at the regular price: USD 38.00
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 26.50