Skip to main content
SpringerLink
Log in

Definition of Conotruncal Anomalies

  • Chapter
  • First Online:
Surgery of Conotruncal Anomalies

Abstract

Conotruncal anomalies are malformations of the infundibulum (conus arteriosus) and great arteries (truncus arteriosus) that have abnormal ventriculo-arterial alignments and connections: tetralogy of Fallot, truncus arteriosus, interrupted aortic arch type B, transposition of the great arteries, double-outlet right ventricle, double-outlet left ventricle, anatomically corrected malposition of the great arteries. Normally related great arteries have complete right-left (R-L) asymmetry in the development and anatomy of the subarterial conal free walls, with involution of the subaortic conal free wall musculature and good growth and expansion of the subpulmonary conal free wall musculature.

All conotruncal anomalies lack this type of complete R-L conal free wall asymmetry. Solitus normally related great arteries (SNRGA) result from involution of the right-sided subaortic conal free wall and from simultaneous good growth and expansion of the left-sided subpulmonary conal free wall. Briefly, \( \mathrm{SNRGA}\kern0.28em =\kern0.28em \mathrm{OR}\kern0.28em +\kern0.28em 4\mathrm{L} \). The right-sided subaortic conal free wall muscular development is grade 0 (absent) and the left-sided subpulmonary conal free wall muscular development is grade 4 (normal). This is how the embryonic aortic switch is normally performed. The left-sided PA is carried superiorly, anteriorly, and rightward above the RV, away from the interventricular foramen, atop the developing subpulmonary conal free wall. Simultaneously, the right-sided Ao moves inferiorly, posteriorly, and leftward because the subaortic conal free wall is undergoing involution, thought to be due to apoptosis. Consequently, the aorta passes mostly through the interventricular foramen and normally comes into direct fibrous continuity with the developing mitral valve (MV), above the morphologically left ventricle (LV), thereby accomplishing the normal embryonic aortic switch. Eleven normal and abnormal developmental and anatomic conotruncal equations are presented in this chapter. From an evolutionary perspective, the conotruncal anomalies appear to represent physiologically unsuccessful attempts to perform the embryonic aortic switch. Conotruncal anomalies are infundibular malformations, causing the great arteries to be abnormally connected, abnormally aligned, and abnormally related in space.

To summarize, the evolution of complete R-L asymmetry in the development of the subarterial conal free walls made possible the embryonic aortic switch. This in turn led to the development of solitus normally related great arteries: \( \mathrm{SNRGA}\kern0.28em =\kern0.28em \mathrm{OR}\kern0.28em +\kern0.28em 4\mathrm{L} \). Its mirror-image isomer is: \( \mathrm{INRGA}\kern0.28em =\kern0.28em 4\mathrm{R}\kern0.28em +\kern0.28em 0\mathrm{L} \). Any other pattern of conal development results in a conotruncal malformation.

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 189.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 249.00
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 249.00
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

Similar content being viewed by others

Notes

  1. 1.

    Ao, aorta; PA, main pulmonary artery; Rv, rotation of semilunar valves (V) relative to the anteroposterior (AP) plane; RAo & PA rotation of the aortic arch and pulmonary bifurcation relative to the AP plane; measurements in degrees (o).

  2. 2.

    Situs is both singular and plural. Situs, sitūs is a fourth declension Latin masculine noun.

References

  1. Van Praagh R, Van Praagh S. Isolated ventricular inversion. A consideration of the morphogenesis, definition, and diagnosis of nontransposed and transposed great arteries. Am J Cardiol. 1966;17:395–406.

    Article  PubMed  Google Scholar 

  2. Van Praagh R. The cardiovascular keys to air-breathing and permanent land-living in vertebrates: the normal human embryonic aortic switch procedure produced by complete right-left asymmetry in the development of the subarterial conal free walls, and the evolution of the right ventricular sinus. Kardiochirurgia i Torakochirurgia Polska. 2011;8:1–22.

    Google Scholar 

  3. Van Praagh R. The importance of segmental situs in the diagnosis of congenital heart disease. Semin Roentgenol. 1985;20:254–71.

    Article  PubMed  Google Scholar 

  4. Taussig HB, Bing RJ. Complete transposition of the aorta and a levoposition of the pulmonary artery: clinical, physiological, and pathological findings. Am Heart J. 1949;37:551–9.

    Article  CAS  PubMed  Google Scholar 

  5. Van Praagh R. What is the Taussig-Bing malformation? Circulation. 1968;38:445–9.

    Article  PubMed  Google Scholar 

  6. Paul MH, Muster AJ, Sinha SN, Cole RB, Van Praagh R. Double-outlet left ventricle with an intact ventricular septum. Clinical and autopsy diagnosis and developmental implications. Circulation. 1970;41:129–39.

    Article  CAS  PubMed  Google Scholar 

  7. Van Praagh R, Van Praagh S. The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications. A study of 57 necropsy cases. Am J Cardiol. 1965;16:406–25.

    Article  PubMed  Google Scholar 

  8. Vizcaino A, Campbell J, Litovsky S, Van Praagh R. Single origin of right and left pulmonary artery branches from ascending aorta with nonbranching main pulmonary artery: relevance to a new understanding of truncus interiosus. Pediatr Cardiol. 2002;23:230–4.

    Article  CAS  PubMed  Google Scholar 

  9. Van Praagh R, Durnin RE, Jockin H, Wagner HR, Korns M, Garbedian H, Ando M, Calder AL. Anatomically corrected malposition of the great arteries {S,D,L}. Circulation. 1975;51:20–31.

    Google Scholar 

  10. Kreutzer J, Van Praagh R. Comparison of left ventricular outflow tract obstruction in interruption of the aortic arch and in the coarctation of the aorta, with diagnostic, developmental, and surgical implications. Am J Cardiol. 2000;86:856–62.

    Article  CAS  PubMed  Google Scholar 

  11. Van Praagh R. The evolution of the human heart and its relevance to congenital heart disease. Kardiochirurgia i Torakochirurgia Polska. 2011;8(4):427–31.

    Google Scholar 

  12. Bajolle F, Zaffran S, Kelly RG, Hadchouel J, Bonnet D, Brown NA, Buckingham ME. Rotation of the myocardial wall of the outflow tract is implicated in the normal positioning of the great arteries. Circ Res. 2006;98:421–8.

    Article  CAS  PubMed  Google Scholar 

  13. Quain R: Cited by Peacock TB: On malformations, etc. of the human heart, with original cases. London: Churchill; 1858. p. 116. Birmingham, Alabama, The Classics of Medicine Library, 1981, Gryphon Editions Limited.

    Google Scholar 

  14. Foran RB, Belcourt C, Nanton MA, Murphy DA, Weinberg AG, Liebman J, Castañeda AR, Van Praagh R. Isolated infundibuloarterial inversion {S, D, I}: a newly recognized form of congenital heart disease. Am Heart J. 1988;116:1337–50.

    Article  CAS  PubMed  Google Scholar 

  15. Houyel L, Van Praagh R, Lacour-Gayet F, Serraf A, Petit J, Bruniaux J, Planché C. Transposition of the great arteries {S, D, L}: pathologic anatomy, diagnosis, and surgical management of a newly recognized complex. J Thorac Cardiovasc Surg. 1995;110:613–24.

    Article  CAS  PubMed  Google Scholar 

  16. Asami I. Partitioning of the arterial end of the human heart. In: Van Praagh R, Takao A, editors. Etiology and Morphogenesis of Congenital Heart Disease. Mt. Kisco/New York: Futura Publishing Co; 1980. p. 51–61.

    Google Scholar 

Download references

Acknowledgement

I would like to thank Paul M. Weinberg, M.D. for his help in simplifying the notation of the infundibularterial equations.

Author information

Authors and Affiliations

  1. Harvard Medical School, Boston, MA, 02115, USA

    Richard Van Praagh MD, FACC, FAHA, AM(Hon), DrMedHC (Professor of Pathology Emeritus)

  2. Departments of Pathology, Cardiology, and Cardiac Surgery, Boston Children’s Hospital, 300 Longwood Avenue, Boston, MA, 02115, USA

    Richard Van Praagh MD, FACC, FAHA, AM(Hon), DrMedHC (Professor of Pathology Emeritus)

Authors
  1. Richard Van Praagh MD, FACC, FAHA, AM(Hon), DrMedHC
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Richard Van Praagh MD, FACC, FAHA, AM(Hon), DrMedHC .

Editor information

Editors and Affiliations

  1. Royal Brompton Hospital, London, United Kingdom

    Francois Lacour-Gayet

  2. C.S. Mott Children’s Hospital, University of Michigan, Ann Arbor, Michigan, USA

    Edward L. Bove

  3. German Pediatric Heart Center, Asklepios Klinik Sankt Augustin Gmb, Sankt Augustin, Germany

    Viktor Hraška

  4. University of Pittsburgh Medical School, Pittsburgh, Pennsylvania, USA

    Victor O. Morell

  5. Department of Cardiothoracic Surgery, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA

    Thomas L. Spray

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Van Praagh, R. (2016). Definition of Conotruncal Anomalies. In: Lacour-Gayet, F., Bove, E., Hraška, V., Morell, V., Spray, T. (eds) Surgery of Conotruncal Anomalies. Springer, Cham. https://doi.org/10.1007/978-3-319-23057-3_1

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-23057-3_1

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-23056-6

  • Online ISBN: 978-3-319-23057-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation