Hostname: page-component-76fb5796d-skm99 Total loading time: 0 Render date: 2024-04-25T21:03:17.800Z Has data issue: false hasContentIssue false
  • English
  • Français

Discordant atrioventricular and ventriculoarterial connections with partially unguarded left-sided atrioventricular orifice and aortic atresia

Published online by Cambridge University Press:  19 August 2008

Matthias Freund ,
Achim Magener  and
Klaus G. Schmidt
Matthias Freund
Affiliation:
From the Department of Pediatric Cardiology, University Children's Hospital, University of Heidelberg, Germany
Achim Magener
Affiliation:
From the Department of Pediatric Cardiology, The Institute of Pathology, University of Heidelberg, Germany
Klaus G. Schmidt*
Affiliation:
From the Department of Pediatric Cardiology, University Children's Hospital, University of Heidelberg, Germany
*
Klaus G. Schmidt, M.D. Department of Pediatric Cardiology, University Children's Hospital, Im Neuenheimer Feld 150, 69120 Heidelberg, F.R.G. Tel. 49-6221-562311, Fax 49-6221-565790
Get access Rights & Permissions [Opens in a new window]

Abstract

Congenitally unguarded morphologically tricuspid valvar orifice is a rare abnormality, usually associated with pulmonary atresia. We describe such a case found in association with discordant atrioventricular and ventriculoarterial connections in which the aortic valve was atretic. Presentation was with respiratory distress due to severe cardiac failure after birth. Echocardiography demonstrated a dilated systemic ventricle with poor function, left atrial enlargement, and aortic atresia. The clinical findings were confirmed at autopsy.

Keywords

Unguarded tricuspid valvar orificeaortic valvar atresiaEbstein's malformation
Type
Brief-Reports
Information
Cardiology in the Young , Volume 7 , Issue 4 , October 1997 , pp. 450 - 453
Copyright
Copyright © Cambridge University Press 1997

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.Kanjuh, VI,Stevenson, JE,Amplatz, K,Edwards, JE. Congenitally unguarded tricuspid orifice with coexistent pulmonary atresia. Circulation 1964; 30:911–917CrossRefGoogle ScholarPubMed
2.Anderson, RH, Silverman, NH, Zuberbuhler, JR. Congenitally unguarded tricuspid valve orifice: Its differentiation from Ebstein's malformation in association with pulmonary atresia and intact ventricular septum.Pediatr Cardiol 1990; 11:86–90.CrossRefGoogle ScholarPubMed
3.Celermajer, JM,Cullen, S, Deanfield, JE,Sullivan, ID. Congenitally corrected transposition and Ebstein's anomaly of the systemic atrioventricular valve: Association with aortic arch obstruction. J Am Coll Cardiol 1991; 18:1056–1058.CrossRefGoogle ScholarPubMed
4.Brenner, JI, Bharati, S, Winn, WC, Lev, M. Absent tricuspid valve with aortic atresia in mixed levocardia (atrial situs solitus, 1-loop). Circulation 1978; 57:836–840.CrossRefGoogle Scholar
5.Gussenhoven, EJ, Essed, CE, Bos, E. Unguarded tricuspid valve orifice with two-chambered right ventricle. Pediatr Cardiol 1986; 7:175–177.CrossRefGoogle ScholarPubMed
6.Leung, MP, Baker, EJ, Anderson, RH, Zuberbuhler, JR. Cineangiographic spectrum of Ebstein's malformation: its relevance to clinical presentation and outcome. J Am Coll Cardiol 1988; 11:154–161.CrossRefGoogle ScholarPubMed
7.Roberson, DA, Silverman, NH. Ebstein's anomaly: echocardiographic and clinical features in the fetus and neonate. J Am Coll Cardiol 1989;14:1300–1307.CrossRefGoogle ScholarPubMed
8.Becker, AE, Becker, MJ, Edwards, JE. Pathologic spectrum of dysplasia of the tricuspid valve. Arch Path 1971; 91:167–178.Google ScholarPubMed
9.Hornberger, LK, Sahn, DJ, Kleinman, CS, Copel, JA, Reed, KL. Tricuspid valve disease with significant tricuspid insufficiency in the fetus: diagnosis and outcome. J Am Coll Cardiol 1991; 17:167–173.CrossRefGoogle ScholarPubMed
10.Silverman, NH, Schmidt, KG. Ventricular volume overload in the human fetus: observations from fetal echocardiography. J Am Soc Echo 1990; 3:20–29.CrossRefGoogle ScholarPubMed