Hostname: page-component-cd9895bd7-q99xh Total loading time: 0 Render date: 2024-12-27T22:02:17.708Z Has data issue: false hasContentIssue false
  • English
  • Français

Congenitally corrected transposition presenting at 24 years: errors of management

Published online by Cambridge University Press:  19 August 2008

L. M. Gerlis ,
R. Egdell  and
Jane Somerville
L. M. Gerlis*
Affiliation:
Grown Up Congenital Heart Unit and Department of Paediatrics, The Royal Brompton Hospital, London, UK
R. Egdell
Affiliation:
Grown Up Congenital Heart Unit and Department of Paediatrics, The Royal Brompton Hospital, London, UK
Jane Somerville
Affiliation:
Grown Up Congenital Heart Unit and Department of Paediatrics, The Royal Brompton Hospital, London, UK
*
L.M. Gerlis, Paediatric, Imperial College School of Medicine, at National Heart & Lung Institute, Dovehouse Street, London SW3 6LY, UK. Tel: 0171 351 8752; Fax 0171 351 8230.
Get access Rights & Permissions [Opens in a new window]

Abstract

A 24-year-old female without previous symptoms or known heart disease consulted a general practitioner with dyspnoea which had slowly worsened over a period of two years. She was treated for asthma, without investigations, until she presented with rapid atrial fibrillation, chest pain and pulmonary oedema necessitating admission to hospital, where she was found to have “mitral” regur-gitation (of the left-sided atrioventricular valve) and congenitally corrected transposition. At operation the grossly regurgitant left-sided morphologically tricuspid valve was replaced by a “top-hat” inverted aortic valve homograft. The procedure resulted in intermittent heart block, needing a pacemaker, and continued myocardial failure which progressed with new right-sided valvar regurgitation. She died a few months later. Post mortem examination confirmed the double discordance, and showed a fistula around the tricuspid valvar prosthesis, left atrial thrombus and changes in the mitral valve. There was also an unsuspected area of old myocardial ischaemia, associated with severe hypoplasia of the circumflex branch of the right-sided coronary artery. This was considered probably to have been of embolic origin.

Keywords

Congenitally corrected transpositiontricuspid regurgitationmitral regurgitationischaemic heart diseasecoronary arterial embolismleft atrioventricular valvar replacement
Type
Clinico-Pathological Correlation
Information
Cardiology in the Young , Volume 7 , Issue 4 , October 1997 , pp. 465 - 470
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.Presbitero, P, Somerville, J, Rabajoli, F, Stone, S, Conte, MR. Corrected transposition of the great arteries without associated defects in adult patients: Clinical profile and follow up. Br Heart J 1995; 74: 57–59.CrossRefGoogle ScholarPubMed
2.Benson, LN, Burns, R, Schwaiger, M, et al. . Radionuelide angiographic evaluation of ventricular function in isolated congenitally corrected transposition of the great arteries. Am J Cardiol 1986;58:319 324.CrossRefGoogle ScholarPubMed
3.Dinas, AP, Moodie, DS, Sterba, R, Gill, CS. Long term function of the morphologically right ventricle in adult patients with corrected transposition of the great arteries. Am Heart J 1989; 118:526–530.CrossRefGoogle Scholar
4.Peterson, RJ, Franch, RH, Fajman, WA, Jones, RH. Comparison of cardiac function in surgically corrected and congenitally corrected transposition of the great arteries. J Thorac Cardiovasc Surg 1988;96:227–236.CrossRefGoogle ScholarPubMed
5.Lieberson, AD, Scliumacher, RR, Childress, RH, Genovese, PD. Corrected transposition of the great vessels in a 73 year old man. Circulation 1969:39;96–100.CrossRefGoogle ScholarPubMed
6.Losekoot, TG, Anderson, RH, Becker, AE, Danielson, GK, Soto, B. Congenitally corrected transposition. Churchill Livingstone: Edinburgh, 1983 p101.Google Scholar
7.Gerlis, LM, Wilson, N, Dickinson, DF. Abnormalities of the mitral valve in congenitally corrected transposition (discordant atrioventricular and ventriculo-arterial connections). Br Heart J 1986;55:475–479.CrossRefGoogle Scholar