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Cor triatriatum or divided atriums: which approach provides the better understanding?

Published online by Cambridge University Press:  13 May 2014

Tara Bharucha ,
Diane E. Spicer ,
Timothy J. Mohun ,
David Black ,
G. William Henry  and
Robert H. Anderson
Tara Bharucha
Affiliation:
Department of Paediatric Cardiology, University Hospital Southampton NHS Foundation Trust, United Kingdom
Diane E. Spicer
Affiliation:
Department of Pediatric Cardiology, University of Florida, Gainesville, Florida, United States of America Congenital Heart Institute of Florida, St Petersburg, Florida, United States of America
Timothy J. Mohun
Affiliation:
Division of Developmental Biology, MRC National Institute for Medical Research, London, United Kingdom
David Black
Affiliation:
Department of Paediatric Cardiology, University Hospital Southampton NHS Foundation Trust, United Kingdom
G. William Henry
Affiliation:
Emeritus Founding Editor, Chapel Hill, North Carolina, United States of America
Robert H. Anderson*
Affiliation:
Department of Paediatric Cardiology, University Hospital Southampton NHS Foundation Trust, United Kingdom Institute of Genetic Medicine, Newcastle University, Newcastle, United Kingdom
*
Correspondence to: Professor R. H. Anderson, 60 Earlsfield Road, London SW18 3DN, United Kingdom. Tel: +00-44-20-8870-4368; E-mail: sejjran@ucl.ac.uk
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Abstract

It is frequent, in the current era, to encounter congenital cardiac malformations described in terms of “cor triatriatum”. But can hearts be truly found with three atrial chambers? The morphological method, emphasised by Van Praagh et al, states that structures within the heart should be defined on the basis of their most constant components. In the atrial chambers, it is the appendages that are the most constant components, and to the best of our knowledge, hearts can only possess two appendages, which can be of either right or left morphology. The hearts described on the basis of “cor triatriatum”, nonetheless, can also be analysed on the basis of division of either the morphologically right or the morphologically left atriums. In this review, we provide a description of cardiac embryology, showing how each of the atrial chambers possesses part of the embryological body, along with an appendage, a vestibule, a venous component, and a septum that separates them. We then show how it is, indeed, the case that the hearts described in terms of “cor triatriatum” can be readily understood on the basis of division of these atrial components. In the right atrium, it is the venous valves that divide the chamber. In the left atrium, it is harder to provide an explanation for the shelf that produces atrial division. We also contrast the classic examples of the divided atrial chambers with the vestibular shelf that produces supravalvar stenosis in the morphologically left atrium, showing that this form of obstruction needs to be distinguished from the fibrous shelves producing intravalvar obstruction.

Keywords

Embryologypersistent venous valvessupramitral stenosispersistent left superior caval vein
Type
Review Articles
Information
Cardiology in the Young , Volume 25 , Issue 2 , February 2015 , pp. 193 - 207
Copyright
Copyright © Cambridge University Press 2014 

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References

1. Niwayama, G. Cor triatriatum: review. Am Heart J 1960; 59: 291–317.CrossRefGoogle Scholar
2. Ott, DA, Cooley, DA, Angelini, P, Leachman, RD. Successful surgical correction of symptomatic cor triatriatum dexter. J Thorac Cardiovasc Surg 1979; 78: 573–575.CrossRefGoogle ScholarPubMed
3. Ostman-Smith, I, Silverman, NH, Oldershaw, P, Lincoln, C, Shinebourne, EA. Cor triatriatum sinistrum. Diagnostic features on cross sectional echocardiography. Br Heart J 1984; 51: 211–219.Google Scholar
4. Van Praagh, R. The segmental approach to diagnosis in congenital heart disease. In: Bergsma D (ed.). Birth Defects Original Article Series, vol. VIII, No. 5. The National Foundation – March of Dimes.Williams and Wilkins, Baltimore, 1972: 4–23.Google Scholar
5. Van Praagh, R, David, I, Wright, GB, Van Praagh, S. Large RV plus small LV is not single RV. Circulation 1980; 61: 1057–1058.CrossRefGoogle Scholar
6. Abbott, ME. Atlas of Congenital Cardiac Disease. American Heart Association, New York, 1936: 2–3.Google Scholar
7. Mohun, TJ, Weninger, WJ. Imaging heart development using high-resolution episcopic microscopy. Curr Opin Genet Dev 2011; 21: 573–578.CrossRefGoogle ScholarPubMed
8. Moorman, AFM, Christoffels, VM, Anderson, RH, van den Hoff, MJB. The heart-forming fields – one or multiple? Phil Trans R Soc B 2007; 362: 1257–1265.Google Scholar
9. 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.CrossRefGoogle ScholarPubMed
10. Yater, WM. Variations and anomalies of the venous valves of the right atrium of the human heart. Arch Pathol 1929; 7: 418–441.Google Scholar
11. Falcone, MW, Roberts, WC. Atresia of the right atrial ostium of the coronary sinus unassociated with persistence of the left superior vena cava: a clinicopathologic study of four adult patients. Am Heart J 1972; 83: 604–611.CrossRefGoogle ScholarPubMed
12. Trento, A, Zuberbuhler, JR, Anderson, RH, Park, SC, Siewers, RD. Divided right atrium (prominence of the Eustachian and Thebesian valves). J Thorac Cardiovasc Surg 1988; 96: 457–463.CrossRefGoogle ScholarPubMed
13. Mahy, IR, Anderson, RH. Division of the right atrium. Images in cardiovascular medicine. Circulation 1998; 98: 2352–2353.CrossRefGoogle Scholar
14. Church, WS. Congenital malformation of the heart: abnormal septum in left auricle. Trans Pathol Soc Lond 1868; 19: 188–190.Google Scholar
15. Gharagozloo, F, Bulkley, BH, Hutchins, GM. A proposed pathogenesis of cor triatriatum: impingement of the left superior caval vein on the developing left atrium. Am Heart J 1977; 94: 618–626.CrossRefGoogle Scholar
16. Van Praagh, R, Corsini, I. Cor triatriatum: pathologic anatomy and a consideration of morphogenesis based on 13 postmortem cases and a study of normal development of the pulmonary vein and atrial septum in 83 human embryos. Am Heart J 1969; 78: 379–405.Google Scholar
17. Webb, S, Kanani, M, Anderson, RH, Richardson, MK, Brown, NA. Development of the human pulmonary vein and its incorporation in the morphologically left atrium. Cardiol Young 2001; 11: 632–642.CrossRefGoogle ScholarPubMed
18. Webb, S, Brown, NA, Wessels, A, Anderson, RH. Development of the murine pulmonary vein and its relationship to the embryonic venous sinus. Anat Rec 1998; 250: 325–334.Google Scholar
19. Thilenius, OG, Bharati, S, Lev, M. Subdivided left atrium: an expanded concept of cor triatriatum sinistrum. Am J Cardiol 1976; 37: 743–752.CrossRefGoogle ScholarPubMed
20. Toscano, A, Pasquini, L, Iacobelli, R, et al. Congenital supravalvar mitral ring: an underestimated anomaly. J Thorac Cardiovasc Surg 2009; 137: 538–542.Google Scholar
21. Shone, JD, Sellers, RD, Anderson, RC, Adams, P, Lillehei, CW, Edwards, JE. The developmental complex of “parachute mitral valve”, supravalvular ring of the left atrium, subaortic stenosis and coarctation of the aorta. Am J Cardiol 1963; 11: 714–725.Google Scholar