Hostname: page-component-586b7cd67f-l7hp2 Total loading time: 0 Render date: 2024-11-24T14:31:43.482Z Has data issue: false hasContentIssue false
  • English
  • Français

Problems in the diagnosis of discordant atrioventricular with concordant ventriculo-arterial connections: anatomical considerations, surgical management, and long-term outcome

Published online by Cambridge University Press:  14 September 2015

Daniela Laux ,
Lucile Houyel ,
Fanny Bajolle ,
Francesca Raimondi ,
Younes Boudjemline  and
Damien Bonnet
Daniela Laux
Affiliation:
Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-Necker, Hôpital Necker Enfants Malades, Assistance Publique des Hôpitaux de Paris, Université Paris Descartes, Sorbonne Paris Cité, Paris, France, European Union Centre de Référence Malformations Cardiaques Congénitales Complexes – M3C, Department of Congenital Cardiac Surgery, Centre Chirurgical Marie-Lannelongue, Le Plessis Robinson, France, European Union
Lucile Houyel
Affiliation:
Centre de Référence Malformations Cardiaques Congénitales Complexes – M3C, Department of Congenital Cardiac Surgery, Centre Chirurgical Marie-Lannelongue, Le Plessis Robinson, France, European Union
Fanny Bajolle
Affiliation:
Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-Necker, Hôpital Necker Enfants Malades, Assistance Publique des Hôpitaux de Paris, Université Paris Descartes, Sorbonne Paris Cité, Paris, France, European Union
Francesca Raimondi
Affiliation:
Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-Necker, Hôpital Necker Enfants Malades, Assistance Publique des Hôpitaux de Paris, Université Paris Descartes, Sorbonne Paris Cité, Paris, France, European Union
Younes Boudjemline
Affiliation:
Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-Necker, Hôpital Necker Enfants Malades, Assistance Publique des Hôpitaux de Paris, Université Paris Descartes, Sorbonne Paris Cité, Paris, France, European Union
Damien Bonnet*
Affiliation:
Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-Necker, Hôpital Necker Enfants Malades, Assistance Publique des Hôpitaux de Paris, Université Paris Descartes, Sorbonne Paris Cité, Paris, France, European Union
*
Correspondence to: D. Bonnet, MD, PhD, Necker-M3C, Pediatric Cardiology, Hôpital Necker Enfants Malades, 149 rue de Sèvres, 75015 Paris, France, European Union. Tel: +3 314 449 4344; Fax: +3 314 449 4340; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Background

Discordant atrioventricular with concordant ventriculo-arterial connections is a rare cardiac defect. When isolated, the haemodynamics resemble transposition of the great arteries. In complex heart defects such as heterotaxy, haemodynamics guide the surgical approach.

Objective

To report a series of eight patients with discordant atrioventricular and concordant ventriculo-arterial connections focussing on anatomical and diagnostic difficulties, surgical management, and follow-up.

Methods

A retrospective review was carried out from 1983 to 2013. Anatomical description was based on segmental analysis. Emphasis was placed on the venoatrial connections.

Results

Segmental arrangement was {I, D, S} in six patients, all with spiralling great vessels. There were two patients with parallel great vessels of whom one had {S, L, D} and the other had {S, L, A} arrangement. Of eight patients, five had heterotaxy syndrome. Median age at repair surgery was 1.4 years (with a range from 1.1 months to 8.1 years). The repair surgery finally performed was the atrial switch procedure in seven out of eight patients. The main post-operative complications were two cases of baffle obstruction and one sick sinus syndrome needing pacemaker implantation. There were two early post-operative deaths and six late survivors. Median follow-up was 4.2 years (with a range from 3.9 to 26.7 years) with good functional status in all survivors.

Discussion

Diagnosing discordant atrioventricular with concordant ventriculo-arterial connections remains challenging. There are ongoing controversies about the definition of atrial morphology and heterotaxy syndrome animating the anatomic discussion of these complex heart defects. Haemodynamically, the atrial switch procedure is the surgical method of choice with an encouraging long-term follow-up despite rhythm disturbances and baffle obstruction.

Keywords

Discordant atrioventricular connectionconcordant ventriculo-arterial connectionatrial switchheterotaxyleft isomerism
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Supplement 1 , January 2016 , pp. 127 - 138
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. Pasquini, L, Sanders, SP, Parness, I, et al. Echocardiographic and anatomic findings in atrioventricular discordance with ventriculoarterial concordance. Am J Cardiol 1988; 1 (62): 1256–1262.Google Scholar
2. Van Praagh, R, Van Praagh, S. Isolated ventricular inversion. A consideration of the morphogenesis, definition and diagnosis of non transposed and transposed great arteries. Am J Cardiol 1966; 17: 395–406.Google Scholar
3. Espino-Vela, J, De la Cruz, MV, Muñoz-Castellanos, L, Plaza, L, Attie, F. Ventricular inversion without transposition of the great vessels in situs inversus. Br Heart J 1970; 32: 292–303.Google Scholar
4. Ostermeyer, J, Bircks, W, Krian, A, Sievers, G, Hilgenberg, F. Isolated atrioventricular discordance. Report of two surgical cases with isolated ventricular inversion. J Thorac Cardiovasc Surg 1983; 86: 926–929.Google Scholar
5. Ranjit, MS, Wilkinson, JL, Mee, RB. Discordant atrioventricular connexion with concordant ventriculo-arterial connexion (so-called “isolated ventricular inversion”) with usual atrial arrangement (situs solitus). Int J Cardiol 1991; 31: 114–117.Google Scholar
6. McElhinney, DB, Reddy, VM, Silverman, NH, Hanley, FL. Intraatrial baffle repair of isolated ventricular inversion with left atrial isomerism. Ann Thorac Surg 1996; 62: 1529–1532.Google Scholar
7. Konstantinov, IE, Lai, L, Colan, SD, et al. Atrioventricular discordance with ventriculoarterial concordance: a remaining indication for the atrial switch operation. J Thorac Cardiovasc Surg 2004; 128: 944–945.Google Scholar
8. Kannan, BR, Kamath, KP, Rao, SG, Kumar, RK. Successful management of isolated ventricular inversion in an adult. Pediatr Cardiol 2004; 25: 554–557.CrossRefGoogle ScholarPubMed
9. Van Praagh, R. Nomenclature and classification: morphologic and segmental approach to diagnosis. In Moller JH, Hoffman JIE (eds). Pediatric Cardiovascular Medicine. Churchill Livingstone, New York, 2000: 275–288.Google Scholar
10. Van Praagh, S, Kakou-Guikahue, M, Kim, HS, Becker, J, Alday, L, Van Praagh, R. Atrial situs in patients with visceral heterotaxy and congenital heart disease: conclusions based on findings in 104 post-mortem cases. Coeur 1988; 19: 484–502.Google Scholar
11. Jacobs, JP, Anderson, RH, Weinberg, PM, et al. The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young 2007; 17 (Suppl 2): 1–28.Google ScholarPubMed
12. 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
13. Sharma, R, Marwah, A, Shah, S, Maheshwari, S. Isolated atrioventricular discordance: surgical experience. Ann Thorac Surg 2008; 85: 1403–1406.CrossRefGoogle ScholarPubMed
14. Snider, AR, Enderlein, MA, Teitel, DF, Hirji, M, Heymann, MA. Isolated ventricular inversion: two-dimensional echocardiographic findings and a review of the literature. Pediatr Cardiol 1984; 5: 27–33.Google Scholar
15. Uemura, H, Ho, SY, Devine, WA, Kilpatrick, LL, Anderson, RH. Atrial appendages and venoatrial connections in hearts from patients with visceral heterotaxy. Ann Thorac Surg 1995; 60: 561–569.Google Scholar
16. Bamforth, SD, Braganca, J, Farthing, CR, et al. Cited-2 controls left-right patterning and heart development through a Nodal-Pitx2c pathway. Nat Genet 2004; 36: 1189–1196.Google Scholar
17. Hildreth, V, Webb, S, Chaudhry, B, et al. Left cardiac isomerism in Sonic hedgehog null mouse. J Anat 2009; 214: 894–904.Google ScholarPubMed
18. Anderson, C, Devine, WA, Anderson, RH, Debich, DE, Zuberbuhler, JR. Abnormalities of the spleen in relation to congenital malformations of the heart: a survey of necropsy findings in children. Br Heart J 1990; 63: 122–128.Google Scholar
19. 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.Google Scholar
20. Van Praagh, R, Perez-Trevino, C, Lopez-Cuellar, M, et al. Transposition of the great arteries with posterior aorta, anterior pulmonary artery, subpulmoanry conus and fibrous continuity between aortic and atrioventricular valves. Am J Cardiol 1971; 28: 621–631.Google Scholar
21. Anderson, RH, Becker, AE, Losekoot, TG, Gerlis, LM. Anatomically corrected malposition of the great arteries. B Heart J 1975; 37: 993–1013.Google Scholar
22. Clarkson, PM, Brandt, PW, Barrat-Boyes, BG, Neutze, JM. “Isolated atrial inversion”. Visceral situs solitus, visceroatrial discordance, discordant ventricular d loop without transposition, dextrocardia: diagnostic and surgical correction. Am J Cardio 1972; 29: 877–881.Google Scholar
23. Moons, P, Gewillig, M, Sluysmans, T, et al. Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium. Heart 2004; 90: 307–313.Google Scholar
24. Dos, L, Teruel, L, Ferreira, IJ, et al. Late outcome of Senning and Mustard procedures for correction of transposition of the great arteries. Heart 2005; 91: 652–656.Google Scholar
25. Warnes, CA. Transposition of the great arteries. Circulation 2006; 114: 2699–2709.Google Scholar
26. McGrath, LB, Kirklin, JW, Blackstone, EH, et al. Death and other events after cardiac repair in discordant atrioventricular connection. J Thorac Cardiovasc Surg 1985; 90: 711–728.Google Scholar
27. Dick, M 2nd, Van Praagh, R, Rudd, M, Folkerth, T, Castaneda, AR. Electrophysiological delineation of the specialized atrioventricular conduction system in two patients with corrected transposition of the great arteries in situs inversus (I,D,D). Circulation 1977; 55: 896–900.Google Scholar
28. Thiene, G, Nava, A, Rossi, L. The conduction system in corrected transposition with situs inversus. Eur J Cardiol 1977; 6: 57–70.Google Scholar
29. Wilkinson, JL, Smith, A, Lincoln, C, Anderson, RH. Conducting tissues in congenitally corrected transposition with situs inversus. Br Heart J 1978; 40: 41–48.Google Scholar
30. Hosseinpour, AR, McCarthy, KP, Griselli, M, Sethia, B, Ho, SY. Congenitally corrected transposition: size of the pulmonary trunk and septal malalignment. Ann Thorac Surg 2004; 77: 2163–2166.Google Scholar
31. de Leval, MR, Bastos, P, Stark, J, Taylor, JF, Macartney, FJ, Anderson, RH. Surgical technique to reduce the risks of heart block following closure of ventricular septal defect in atrioventricular discordance. J Thorac Cardiovasc Surg 1979; 78: 515–526.Google Scholar
Supplementary material: Image

Laux supplementary material

Figure S1

Download Laux supplementary material(Image)
Image 4 MB
Supplementary material: Image

Laux supplementary material

Figure S2

Download Laux supplementary material(Image)
Image 2 MB
Supplementary material: Image

Laux supplementary material

Figure S3

Download Laux supplementary material(Image)
Image 1.2 MB
Supplementary material: Image

Laux supplementary material

Figure S4

Download Laux supplementary material(Image)
Image 2 MB
Supplementary material: Image

Laux supplementary material

Figure S5

Download Laux supplementary material(Image)
Image 2.2 MB

Laux supplementary material

Video S1

Download Laux supplementary material(Video)
Video 14.4 MB