Hostname: page-component-788cddb947-pt5lt Total loading time: 0 Render date: 2024-10-08T22:28:01.895Z Has data issue: false hasContentIssue false
  • English
  • Français

Unguarded mitral orifice, mirror-imaged atrial arrangement, and discordant atrioventricular connections

Published online by Cambridge University Press:  19 August 2008

Satoshi Yasukochi ,
Gengi Satomi ,
Insam Park ,
Masahiko Ando  and
Kazuo Momma
Satoshi Yasukochi*
Affiliation:
Department of Pediatric Cardiology, Nagano Children's Hospital, Nagano, Japan
Gengi Satomi
Affiliation:
Department of Pediatric Cardiology, Nagano Children's Hospital, Nagano, Japan
Insam Park
Affiliation:
Department of Pediatric Cardiology, The Heart Institute of Japan, Tokyo Women's Medical College, Tokyo, Japan
Masahiko Ando
Affiliation:
Department of Pediatric Cardiology, The Heart Institute of Japan, Tokyo Women's Medical College, Tokyo, Japan
Kazuo Momma
Affiliation:
Department of Pediatric Cardiology, The Heart Institute of Japan, Tokyo Women's Medical College, Tokyo, Japan
*
Satoshi Yasukochi, M.D., Department of Pediatric Cardiology, Nagano Children's Hospital, 3100 Toyoshina, Toyoshina, Nagano 399–8288, Japan. Tel: +81–263–73–6700; Fax: +81–263–73–5432; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

We report two autopsy proven cases of unguarded mitral orifice associated with mirror-imaged atrial arrangement, discordant atrioventricular connections, double outlet right ventricle, pulmonary valvar stenosis or atresia, and atrialisation of the morphologically left ventricle. The morphologically left atrioventricular junction was devoid of valvar leaflets, and there was no tension apparatus within the ventricle. To the best of our knowledge, this is the first description of this rare cardiac malformation

Keywords

Unguarded mitral orificedouble outlet right ventriclesegmental combination
Type
Original Articles
Information
Cardiology in the Young , Volume 9 , Issue 5 , September 1999 , pp. 478 - 483
Copyright
Copyright © Cambridge University Press 1999

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.Ozkutlu, S, Gunal, N, Caglar, M, Alehan, D, Gungor, C.Unguarded tricuspid orifice: a rare malformation of tricuspid valve diagnosed by echocardiography. Report of two cases and review of the literature. Int J Cardiol 1996;56: 125–129.CrossRefGoogle ScholarPubMed
2.Kanjuh, VI., Stevenson, JE., Amplatz, K, Edwards, JR. Congenitally unguarded tricuspid orifice with coexistent pulmonary atresia. Circulation 1964; 30: 911–917.CrossRefGoogle ScholarPubMed
3.Zuberbuhler, JR, Allwork, SP, and Anderson, RH. The spectrum of Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1979; 77: 202–211.CrossRefGoogle ScholarPubMed
4.Hornberger, LK, Sahn, DJ, Kleinman, CS, Copel, JA, Reed, KL. Tricuspid valve disease with siginificant tricuspid insufficeincy in the fetus: diagnosis and outcome. J Am Coll Cardiol 1991; 17: 167–173.CrossRefGoogle Scholar
5.Anderson, RH, Silverman, NH, Zuberbuhler, JR. Congenitally unguarded tricuspid orifice: its differentiation from Ebstein's malformation in association with pulmonary atresia and intact ventricular septum. Pediatr Cardiol 1990; 11:86–91.CrossRefGoogle ScholarPubMed
6.Freund, M, Magener, A, Schmidt, KG. Discordant atrioventricular and ventriculoarterial connection with partially unguarded left-sided atrioventricular orifice and aortic atresia. Cardiol Young 1997; 7: 450–453.CrossRefGoogle Scholar
7.Uhl, HSM. A previously undescribed congenital malformation of the heart: almost total absence of the myocardium of the right ventricle. Bulletin Johns Hopkins Hospital 1952; 91: 197–209.Google ScholarPubMed
8.Lindinger, A, Masur, Y, Limbach, HG. Absence of the left ventricular myocardium in a newborn. Cardiol Young 1996; 6: 344–347CrossRefGoogle Scholar
9.Ruschhaupt, DG, Bharati, S, Lev, M. Mitral valve malformation of Ebstein type in absence of corrected trasposition. Am J Cardiol 1976; 38(1): 109–112.CrossRefGoogle Scholar
10.Leung, M, Rigby, ML, Anderson, RH, Wyse, RKH, Macartney, FJ. Reversed offsetting of the septal attachments of the atriventricular valves and Ebstein's malformation of the morphologically mitral valve. Br Heart J 1987; 57: 184–187.CrossRefGoogle Scholar
11.Caruso, G, Cifarelli, A, Balduci, G, Facilone, F. Ebstein's maiformation of the mitral valve in atrioventricular and ventriculoarterial concordance. Pediatr Cardiol 1987; 8: 209–210.CrossRefGoogle ScholarPubMed
12.Jacob, JL, Sobrinho, SH, Lorga, AM, Garzon, SA, da-Silveira, LC, Braile, DM. Ebstein's anomaly of the mitral valve. A very unusual congenital malformation of the heart. A case report. Arq Bras Cardiol 1989; 53(5): 283–285.Google ScholarPubMed
13.Dusmet, M, Oberhaensli, I, Cox, JN. Ebstein's anomaly of the tricuspid and mitral valves in an otherwise normal heart. Br Heart J 1987; 58: 400–404.CrossRefGoogle Scholar
14.van Praagh, R, Corwin, RD, Dahlquist, EH Jr, Freedom, RM, Mattioli, L, Nebesar, RA. Tetralogy of Fallot with severe left ventricular outflow tract obstruction due to anomalous attachment of the mitral valve to the ventricular septum. Am J Cardiol 1970; 26: 97–101.CrossRefGoogle Scholar
15.James, TN, Nichols, MM, Sapire, DW, DiPatre, PL, Lopez, SM. Complete heart block and fatal right ventricular failure in an infant. Circulation 1996; 93: 1588–1600.CrossRefGoogle ScholarPubMed
16.Lamers, WH, Viragh, S, Wessels, A, Moorman, AFM, Anderson, RH. Formation of the tricuspid valve in the human heart. Circulation 1995; 91: 111–121.CrossRefGoogle ScholarPubMed