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Successful one stage biventricular correction of aortic atresia with a ventricular septal defect and discordant ventriculo-arterial connections

Published online by Cambridge University Press:  19 August 2008

Tjark Ebels ,
Friedhelm Dapper ,
Jurgen Bauer ,
Rainer M. Bohle ,
Karl J. Hagel ,
Peter G.J. Nikkels  and
Friedrich W. Hehrlein
Tjark Ebels*
Affiliation:
Division of Cardiothoracic Surgery, Thorax Centrum, Groningen, the Netherlands.
Friedhelm Dapper
Affiliation:
Department of Cardiovascular Surgery, Justus-Liebig-Universitat, Giessen, Germany.
Jurgen Bauer
Affiliation:
Department of Paediatric Cardiology, Justus-Liebig-Universitat, Giessen, Germany.
Rainer M. Bohle
Affiliation:
Department of Pathology, Justus-Liebig-Universitat, Giessen, Germany.
Karl J. Hagel
Affiliation:
Department of Paediatric Cardiology, Justus-Liebig-Universitat, Giessen, Germany.
Peter G.J. Nikkels
Affiliation:
Department of Pathology, Academisch Ziekenhuis, Groningen, the Netherlands.
Friedrich W. Hehrlein
Affiliation:
Department of Cardiovascular Surgery, Justus-Liebig-Universitat, Giessen, Germany.
*
Tjark Ebels MD PhD. Division of Cardiothoracic Surgery, Thorax Centrum, Academisch Ziekenhuis, P.O Box 30 001, 9700RB Groningen, The Netherlands. Tel: 31 50 3613238, Fax: 31 50 3611347
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Summary

Aortic atresia is rare in the setting of a normally developed left ventricle with a ventricular septal defect. In this combination, as far as we know, it has been described only with concordant ventriculo-arterial connections, for which seven one-stage biventricular repairs have now been described. We describe here the case of a 3½-month-old male infant with a similar combination but with discordant ventriculo-arterial connections and severe pulmonary hypertension. It was the right ventricle which achieved normal size in this arrangement, presumably because of the ventricular septal defect. One-stage biventricular correction was accomplished, employing a single pulmonary allograft, aided by massive doses of Prostaglandin El. As far as we know this is the first report of the combination of aortic atresia, discordant ventriculo-arterial connections, a ventricular septal defect and balanced ventricles. We complement our surgical account, nonetheless, with a description of a comparable specimen from our anatomic museum.

Keywords

Aortic atresiacomplete transposition
Type
Original Articles
Information
Cardiology in the Young , Volume 7 , Issue 4 , October 1997 , pp. 402 - 409
Copyright
Copyright © Cambridge University Press 1997

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References

1.Freedom, RM, Williams, WG, Dische, MR, Rowe, RD. Anatomical variants in aortic atresia. Potential candidates for ventriculoaortic reconstitution. Br Heart J 1976; 38: 821–826.CrossRefGoogle ScholarPubMed
2.Pellegrino, PA, Thiene, G.Aortic valve atresia with a normally developed left ventricle. Chest 1976; 69: 121–122.CrossRefGoogle ScholarPubMed
3.Roberts, WC, Perry, LW, Chandra, RS, Myers, GS, Shapiro, SR, Scott, LP.Aortic atresia: a new classification based on necropsy study of 73 cases. Am J Cardiol 1976; 37: 753–756.CrossRefGoogle ScholarPubMed
4.Freedom, RM, Dische, MR, Rowe, RD.Conal anatomy of subaortic stenosis and atresia in the first year of life. Am Heart J 1977; 94: 689–698.CrossRefGoogle Scholar
5.Perry, LW, Scott, LP III, Shapiro, SL, Chandra, RS, Roberts, WC. Atresia of the aortic valve with ventricular septal defect. A clinicopathologic study of four newborns. Chest 1977; 72: 757–761.CrossRefGoogle ScholarPubMed
6.Thiene, G, Gallucci, V, Macartney, FJ, Del, Torso S, Pellegrino, PA, Anderson, RH. Anatomy of aortic atresia. Cases presenting with a ventricular septal defect. Circulation 1979; 59: 173–178.CrossRefGoogle ScholarPubMed
7.Imai, Y, Kurosawa, H, Fujiwara, T, Fukuchi, S, Matsuo, K, Kawada, M, Ohtsuka, G.Palliative repair of aortic atresia associated with tricuspid atresia and transposition of the great arteries. Ann Thorac Surg 1991; 51: 646–648.CrossRefGoogle ScholarPubMed
8.McGarry, KM, Taylor, JFN, Macartney, FJ.Aortic atresia occurring with complete transposition of the great arteries. Br Heart J 1980; 44: 711–713.CrossRefGoogle Scholar
9.Mahowald, JM, Lucas, RV, Edwards, JE. Aortic valvular atresia. Associated cardiovascular anomalies. Ped Cardiol 1982; 2: 99–105.CrossRefGoogle ScholarPubMed
10.Austin, EH, Jonas, RA, Mayer, JE, Castaneda, AR. Aortic atresia with normal left ventricle. Single-stage repair in the neonate. J Thorac Cardiovasc Surg 1989; 97: 392–395.CrossRefGoogle ScholarPubMed
11.Bogers, AJJC, Sreeram, N, Hess, J, Sutherland, GR, Quaegebeur, JM.Aortic atresia with normal left ventricle: One-stage repair in early infancy. Ann Thorac Surg 1991; 51: 312–314.CrossRefGoogle ScholarPubMed
12.Serraf, A, Bruniaux, J, Lebidois, J, Lacour-Gayet, F, Kachaner, J, Planché, C.Aortic atresia with normal left ventricle. Ann Thorac Surg 1991; 51: 1017–1019.CrossRefGoogle ScholarPubMed
13.François, K, Dollery, C, Elliott, MJ.Aortic atresia with ventricular septal defect and normal left ventricle: one-stage correction in the neonate. Ann Thorac Surg 1994; 58: 880–882.CrossRefGoogle ScholarPubMed
14.Duffy, CE, Muster, AJ, DeLeon, SY, Idriss, FS, Ilbawi, M, Riggs, TRW, Paul, MH.Successful surgical repair of aortic atresia associated with normal left ventricle. J Am Coll Cardiol 1983; 6: 1503–1506.CrossRefGoogle Scholar
15.Freedom, RM, Culham, JAG, Rowe, RD. Aortic atresia with normal left ventricle. Distinctive angiographic findings. Cathet Cardiovasc Diagn 1977; 3: 283–295.CrossRefGoogle Scholar
16.Freedom, RM, Dische, MR, Rowe, RD. Pathologic anatomy of subaortic stenosis and atresia in the first year of life. Am JGoogle Scholar