Hostname: page-component-8448b6f56d-c47g7 Total loading time: 0 Render date: 2024-04-24T08:34:54.959Z Has data issue: false hasContentIssue false
  • English
  • Français

Clarifying the anatomy of hearts with concordant ventriculo-arterial connections but abnormally related arterial trunks

Published online by Cambridge University Press:  27 August 2015

Saurabh K. Gupta ,
Sivasubramanian Ramakrishnan ,
Gurpreet S. Gulati ,
G. William Henry ,
Diane E. Spicer ,
Carl L. Backer  and
Robert H. Anderson
Saurabh K. Gupta*
Affiliation:
Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India
Sivasubramanian Ramakrishnan
Affiliation:
Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India
Gurpreet S. Gulati
Affiliation:
Department of Cardiac Radiology, All India Institute of Medical Sciences, New Delhi, India
G. William Henry
Affiliation:
Bailliard Henry Pediatric Cardiology, Raleigh, North Carolina, United States of America
Diane E. Spicer
Affiliation:
Division of Pediatric Cardiology, University of Florida College of Medicine, Gainesville, United States of America Congenital Heart Institute of Florida, St. Petersburg, Florida, United States of America
Carl L. Backer
Affiliation:
Division of Cardiovascular-Thoracic Surgery, Department of Pediatrics, Ann and Robert H. Lurie Children’s Hospital of Chicago, Chicago, Illinois, United States of America
Robert H. Anderson
Affiliation:
Institute of Genetic Medicine, University of Newcastle, Newcastle-upon-Tyne, United Kingdom
*
Correspondence to: Dr Saurabh K. Gupta, MD, DM, Assistant Professor, Department of Cardiology, Room No. 23, 7th floor CT Centre, All India Institute of Medical Sciences, New Delhi 110029, India. Tel: +91-11-26594681; Fax: +91-11-26588663; E-mail: drsaurabhmd@gmail.com
Get access Rights & Permissions [Opens in a new window]

Abstract

Hearts in which the arterial trunks arise from the morphologically appropriate ventricles, but in a parallel manner, rather than the usual spiralling arrangement, have long fascinated anatomists. These rare entities, for quite some time, were considered embryological impossibilities, but ongoing experience has shown that they can be found in various segmental combinations. Problems still exist about how best to describe them, as the different variants are often described with esoteric terms, such as anatomically corrected malposition or isolated ventricular inversion. In this review, based on our combined clinical and morphological experience, we demonstrate that the essential feature of all hearts described in this manner is a parallel arrangement of the arterial trunks as they exit from the ventricular mass. We show that the relationship of the arterial roots needs to be described in terms of the underlying ventricular topology, rather than according to the arrangement of the atrial chambers. We then discuss the importance of determining atrial arrangement on the basis of the morphology of the appendages, following the precepts as set out in the so-called “morphological method” and distinguished according to the extent of the pectinate muscles relative to the atrioventricular junctions as opposed to basing diagnosis on the venoatrial connections. We show that, when approached in this manner, the various combinations can be readily diagnosed in the clinical setting and described in straightforward way.

Keywords

Anatomically corrected malpositionisolated ventricular inversionsequential segmental analysisventricular topologyterminology
Type
Review Articles
Information
Cardiology in the Young , Volume 26 , Issue 1 , January 2016 , pp. 1 - 18
Check for updates
Copyright
© Cambridge University Press 2015 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Harris, JS, Farber, S. Transposition of the great cardiac vessels (with special reference to the phylogenetic theory of Spitzer). Arch Path 1938; 28: 427–502.Google Scholar
2. Van Mierop, LHS, Wiglesworth, FW. Pathogenesis of transposition complexes. II Anomalies due to faulty transfer of the posterior great artery. Am J Cardiol 1963; 12: 226–232.CrossRefGoogle Scholar
3. Van Praagh, R, Van Praagh, S. Anatomically corrected transposition of the great arteries. Br Heart J 1967; 29: 112.CrossRefGoogle ScholarPubMed
4. Van Mierop, LHS. Transposition of the great arteries. Clarification or further confusion? Editorial. Am J Cardiol 1971; 28: 735–738.Google Scholar
5. Van Praagh, R. Transposition of the great arteries. II. Transposition clarified. Am J Cardiol 1971; 28: 739–741.CrossRefGoogle ScholarPubMed
6. Kirklin, JW, Pacifico, AD, Bargeron, LM, Soto, B. Cardiac repair in anatomically corrected malposition of the great arteries. Circulation 1973; 48: 153–159.CrossRefGoogle ScholarPubMed
7. Anderson, RH, Becker, AE, Losekoot, TG, Gerlis, LM. Anatomically corrected malposition of the great arteries. Br Heart J 1975; 37: 993–1013.Google Scholar
8. Arciprete, P, Macartey, FJ, de Leva, M, Stark, J. Mustards operation for patients with ventriculoarterial concordance. Br Heart J 1985; 53: 443–450.Google Scholar
9. Van Praagh, R, Van Praagh, S. Isolated ventricular inversion: a consideration of morphogenesis, definition and diagnosis of non-transposed and transposed great arteries. Am J Cardiol 1966; 17: 395–406.Google Scholar
10. Van Praagh, R. Nomenclature and classification: morphologic and segmental approach to diagnosis. In: Moller JH, Hoffman JIE (eds). Chapter 20 in Pediatric Cardiovascular Medicine. Churchill Livingstone, New York, 2000: 282.Google Scholar
11. Anderson, RH, Ho, SY. Continuing medical education. Sequential segmental analysis – description and categorization for the millennium. Cardiol Young 1997; 7: 98–116.Google Scholar
12. Anderson, RH, Shirali, G. Sequential segmental analysis. Ann Pediatr Cardiol 2009; 2: 24–35.Google Scholar
13. Macartney, FJ, Zuberbuhler, JR, Anderson, RH. Morphological considerations pertaining to recognition of atrial isomerism. Consequences for sequential chamber localisation. Br Heart J 1980; 44: 657–667.CrossRefGoogle ScholarPubMed
14. Cavalle-Garrido, T, Bernasconi, A, Perrin, D, Anderson, RH. Hearts with concordant ventriculoarterial connections but parallel arterial trunks. Heart 2007; 93: 100–106.Google Scholar
15. Van Praagh, R, David, I, Wright, GB, Van Praagh, S. Large RV plus small LV is not single RV. Circulation 1980; 61: 1057–1058.Google Scholar
16. Lev, M. Pathologic diagnosis of positional variations in cardiac chambers in congenital heart disease. Lab Invest 1954; 3: 71–82.Google Scholar
17. Uemura, H, Ho, SY, Devine, WA, Kilpatrick, LL, Anderson, RH. Atrial appendages and venoatrial connections in hearts with patients with visceral heterotaxy. Ann Thorac Surg 1995; 60: 561–569.Google Scholar
18. Van Mierop, LHS, Eisen, S, Schiebler, GL. The radiographic appearance of the tracheobronchial tree as an indicator of visceral situs. Am J Cardiol 1970; 26: 432–435.Google Scholar
19. Gupta, SK, Gulati, GS, Saxena, A, Ho, SY, Juneja, R. “Isolated atrial inversion” without transposition physiology: yet another “twisted heart”. World J Ped Congen Heart Surg 2014; 5: 488–490.Google Scholar
20. Seo, J-W, Yoo, S-J, Ho, SY, Lee, HJ, Anderson, RH. Further morphological observations on hearts with twisted atrioventricular connections (criss-cross hearts). Cardiovasc Pathol 1992; 1: 211–217.CrossRefGoogle ScholarPubMed
21. Anderson, RH, Shinebourne, EA, Gerlis, LM. Criss-cross atrioventricular relationships producing paradoxical atrioventricular concordance or discordance. Their significance to nomenclature of congenital heart disease. Circulation 1974; 50: 176–180.CrossRefGoogle ScholarPubMed
22. Sharma, R, Marwah, A, Shah, S, Maheshwari, S. Isolated atrioventricular discordance: surgical experience. Ann Thorac Surg 2008; 85: 1403–1406.CrossRefGoogle ScholarPubMed
23. Ho, SY, Fagg, N, Anderson, RH, Cook, A, Allan, L. Disposition of the atrioventricular conduction tissues in the heart with isomerism of the atrial appendages: its relation to congenital complete heart block. J Am Coll Cardiol 1992; 20: 904–910.CrossRefGoogle ScholarPubMed
24. Smith, A, Ho, SY, Anderson, RH, Connell, MG, Arnold, R, Wilkinson, JL. The diverse cardiac morphology seen in hearts with isomerism of the atrial appendages with reference to the disposition of the specialized conduction system. Cardiol Young 2006; 16: 437–454.Google Scholar
25. Anderson, RH, Brown, NA, Meno, C, Spicer, DE. The importance of being isomeric. Clin Anat 2015; 28: 477–486.Google Scholar