Skip to main content
SpringerLink
Log in

Fetal Pulmonary Valvuloplasty: Natural History and Echocardiographic Evaluation of Critical Pulmonary Stenosis/Pulmonary Atresia with Intact Ventricular Septum

  • Chapter
  • First Online:
Fetal and Hybrid Procedures in Congenital Heart Diseases

  • 783 Accesses

Abstract

Pulmonary atresia with intact ventricular septum (PAIVS) is a morphologically heterogeneous lesion characterized by variable dimensions of the right ventricle (RV) from a normal size to a variable degree of hypoplasia, often with associated anomalies of the coronary circulation. Critical pulmonary stenosis (CPS), also, is often present with an abnormal right ventricle.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 149.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 199.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 199.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Notes

  1. 1.

    Z-scores representing the number of standard deviations with respect to the data of the normal population for a given gestational age, derived from echocardiographic data – Boston criteria, Circulation. 2009;120(15):1482–1490.

References

  1. Bolger AF, Eigler NL, Maurer G. Quantifying valvular regurgitation. Limitations and inherent assumptions of Doppler techniques. Circulation. 1988;78:1316–8.

    Article  CAS  PubMed  Google Scholar 

  2. Rivera JM, Vandervoort PM, Vazquez de Prada JA, et al. Which physical factors determine tricuspid regurgitation jet area in the clinical setting? Am J Cardiol. 1993;72:1305–9.

    Article  CAS  PubMed  Google Scholar 

  3. Kiserud T, Eik-Nes. SH, Blaas HG, Hellevik LR. Ultrasonographic velocimetry of the fetal ductus venosus. Ultrasound Obstet Gynecol. 1991;338:1412–4.

    CAS  Google Scholar 

  4. DeVore GR, Horenstein J. Ductus venosus index: a method for evaluating right ventricular preload in the second-trimester fetus. Ultrasound Obstet Gynecol. 1993;3:338–42.

    Article  CAS  PubMed  Google Scholar 

  5. Gardiner HM, Belmar C, Tulzer G, Barlow A, Pasquini L, Carvalho JS, Daubeney PE, Rigby ML, Gordon F, Kulinskaya E, Franklin RC. Morphologic and functional predictors of eventual circulation in the fetus with pulmonary atresia or critical pulmonary stenosis with intact septum. J Am Coll Cardiol. 2008;51(13):1299–308.

    Article  PubMed  Google Scholar 

  6. Tan J, Silverman NH, Hoffman JIE, Villegas M, Schmidt KG. Cardiac dimensions determined by cross-sectional echocardiography in the normal human fetus from 18 weeks to term. Am J Cardiol. 1992;70:1459–67.

    Article  CAS  PubMed  Google Scholar 

  7. Hornberger LK, Benacerraf BR, Bromley BS, Spevak PJ, Sanders SP. Prenatal detection of severe right ventricular outflow tract obstruction: pulmonary stenosis and pulmonary atresia. J Ultrasound Med. 1994;13:743–50.

    CAS  PubMed  Google Scholar 

  8. Gardiner HM. Progression of fetal heart disease and rationale for fetal intracardiac interventions. Semin Fetal Neonatal Med. 2005;10:578–85.

    Article  PubMed  Google Scholar 

  9. Shinebourne EA, Rigby ML, Carvalho JS. Pulmonary atresia with intact ventricular septum: from fetus to adult. Heart. 2008;94:1350–7.

    Article  PubMed  Google Scholar 

  10. Sharland GK, Tynan M, Qureshi SA. Prenatal detection and progression of right coronary artery to right ventricle fistula. Heart. 1996;76:79–81.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Salvin JW, McElhinney DB, Colan SD, Gauvreau K, del Nido PJ, Jenkins KJ, Lock JE, Tworetzky W. Fetal tricuspid valve size and growth as predictors of outcome in pulmonary atresia with intact ventricular septum. Pediatrics. 2006;118:e415–20.

    Article  PubMed  Google Scholar 

  12. Maeno YV, Boutin C, Hornberger LK, McCrindle BW, Cavallé-Garrido T, Gladman G, Smallhorn JF. Prenatal diagnosis of right ventricular outflow tract obstruction with intact ventricular septum, and detection of ventriculocoronary connections. Heart. 1999;81:661–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Giglia TM, Mandell VS, Connor AR, Mayer JE, Lock JE. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation. 1992;86:1516–28.

    Article  CAS  PubMed  Google Scholar 

  14. Calder AL, Co EE, Sage MD. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum. Am J Cardiol. 1987;59:436–42.

    Article  CAS  PubMed  Google Scholar 

  15. Powell AJ, Mayer JE, Lang P, Lock JE. Outcome in infants with pulmonary atresia, intact ventricular septum, and right ventricle-dependent coronary circulation. Am J Cardiol. 2000;86:1272–4.

    Article  CAS  PubMed  Google Scholar 

  16. Guleserian KJ, Armsby LB, Thiagarajan RR, del Nido PJ, Mayer Jr JE. History of pulmonary atresia with intact ventricular septum and right-ventricle-dependent coronary circulation managed by the single-ventricle approach. Ann Thorac Surg. 2006;81:2250–8.

    Article  PubMed  Google Scholar 

  17. Cha HH, Choo YS, Seong WJ. Prenatal diagnosis of multiple ventriculocoronary connections in pulmonary atresia with an intact ventricular septum: a case report. J Obstet Gynaecol Res. 2015;41:1278–81.

    Article  PubMed  Google Scholar 

  18. Chaoui R, Tennstedt C, Goldner B, Bollmann R. Prenatal diagnosis of ventriculo-coronary communications in second-trimester fetus using transvaginal and transabdominal color Doppler sonography. Ultrasound Obstet Gynecol. 1997;9:194–7.

    Article  CAS  PubMed  Google Scholar 

  19. Sandor GG, Cook AC, Sharland GK, Ho SY, Potts JE, Anderson RH. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum diagnosed during fetal life. Cardiol Young. 2002;12:436–44.

    Article  PubMed  Google Scholar 

  20. Daubeney PEF, Delany DJ, Anderson RH, et al. Pulmonary atresia with intact ventricular septum: range of morphology in a population-based study. J Am Coll Cardiol. 2002;39:1670–9.

    Article  PubMed  Google Scholar 

  21. Vesel S, Rollings S, Jones A, Callaghan N, Simpson J, Sharland GK. Prenatally diagnosed pulmonary atresia with ventricular septal defect: echocardiography, genetics, associated anomalies and outcome. Heart. 2006;92:1501–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Todros T, Presbitero P, Gagliotti P, Demarie D. Pulmonary stenosis with intact ventricular septum: documentation of development of the lesion echocardiographically during fetal life. Int J Cardiol. 1988;19:355–60.

    Article  CAS  PubMed  Google Scholar 

  23. Song TB, Lee JY, Kim YH, Oh BS, Kim EK. Prenatal diagnosis of severe tricuspid insufficiency in Ebstein’s anomaly with pulmonary atresia and intact ventricular septum: a case report. J Obstet Gynaecol Res. 2000;26:223–6.

    Article  CAS  PubMed  Google Scholar 

  24. Mallula K, Vaughn G, El-Said H, Lamberti JJ, Moore JW. Comparison of ductal stenting versus surgical shunts for palliation of patients with pulmonary atresia and intact ventricular septum. Catheter Cardiovasc Interv. 2015;85:1196–202.

    Article  PubMed  Google Scholar 

  25. Huang SC, Ishino K, Kasahara S, Yoshizumi K, Kotani Y, Sano S. The potential of disproportionate growth of tricuspid valve after decompression of the right ventricle in patients with pulmonary atresia and intact ventricular septa. J Thorac Cardiovasc Surg. 2009;138:1160–6.

    Article  PubMed  Google Scholar 

  26. Yoshimura N, Yamaguchi M. Surgical strategy for pulmonary atresia with intact ventricular septum: initial management and definitive surgery. Gen Thorac Cardiovasc Surg. 2009;57:338–46.

    Article  PubMed  Google Scholar 

  27. Waldman JD, Karp RB, Lamberti JJ, Sand ME, Ruschhaupt DG, Agarwala B. Tricuspid valve closure in pulmonary atresia with important RV-to-coronary artery connections. Ann Thorac Surg. 1995;59:933–41.

    Article  CAS  PubMed  Google Scholar 

  28. Iacobelli R, Pasquini L, Toscano A, Raimondi F, Michielon G, Tozzi AE, Sanders SP. Role of tricuspid regurgitation in fetal echocardiographic diagnosis of pulmonary atresia with intact ventricular septum. Ultrasound Obstet Gynecol. 2008;32:31–5.

    Article  CAS  PubMed  Google Scholar 

  29. Peterson RE, Levi DS, Williams RJ, Lai WW, Sklansky MS, Drant S. Echocardiographic predictors of outcome in fetuses with pulmonary atresia with intact ventricular septum. J Am Soc Echocardiogr. 2006;19:1393–400.

    Article  PubMed  Google Scholar 

  30. Roman KS, Fouron JC, Nii M, Smallhorn JF, Chaturvedi R, Jaeggi ET. Determinants of outcome in fetal pulmonary valve stenosis or atresia with intact ventricular septum. Am J Cardiol. 2007;99:699–703.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Center of Fetal Cardiology, Policlinico San Donato IRCCS, Milan, Italy

    Vlasta Fesslova

  2. Pediatric Unit, Department of the Mother and Child Health, IRCCS Policlinico San Matteo Foundation, Pavia, Italy

    Savina Mannarino

Authors
  1. Vlasta Fesslova
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Savina Mannarino
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Vlasta Fesslova .

Editor information

Editors and Affiliations

  1. IRCCS Policlinico San Donato, San Donato Milanese, Italy

    Gianfranco Butera

  2. Pediatric Cardiology, Nationwide Children's Hospital, Columbus, Ohio, USA

    John Cheatham

  3. Instituto Dante Pazzanese de Cardiologia and Hospital do Coração, São Paulo, SP, Brazil, Sao Paulo, Brazil

    Carlos AC Pedra

  4. Giessen Hospital, Giessen, Hessen, Germany

    Dietmar Schranz

  5. Linz University, Linz, Austria

    Gerald Tulzer

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Fesslova, V., Mannarino, S. (2016). Fetal Pulmonary Valvuloplasty: Natural History and Echocardiographic Evaluation of Critical Pulmonary Stenosis/Pulmonary Atresia with Intact Ventricular Septum. In: Butera, G., Cheatham, J., Pedra, C., Schranz, D., Tulzer, G. (eds) Fetal and Hybrid Procedures in Congenital Heart Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-40088-4_10

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-40088-4_10

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-40086-0

  • Online ISBN: 978-3-319-40088-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation