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Dynamic outflow tract obstruction in congenitally corrected transposition of the great arteries

  • Andrew O. Zurick III
  • Venu MenonEmail author
Case in Point

Abstract

Congenitally corrected transposition of the great arteries (CCTGA) is a rare form of congenital heart disease, constituting 0.5% of all congenital heart defects. The incidence of left ventricle (non-systemic ventricle) outflow tract obstruction ranges between 44 and 57%. Herein, we present the case of a 45 year old woman with CCTGA with progressively worsening dyspnea who had been referred for surgical correction of severe systemic ventricle (morphologic right ventricle) atrio-ventricular valve (tricuspid valve) regurgitation. Cardiac magnetic resonance imaging (CMR) and transesophageal imaging (TEE) demonstrated severe systemic ventricle (morphologic right ventricle) contractile dysfunction, as well as dynamic non-systemic ventricle (morphologic left ventricle) outflow tract obstruction due to systolic anterior motion (SAM) of the non-systemic ventricle (morphologic left ventricle) atrio-ventricular valve (mitral valve) with a large membranous ventricular septal aneurysm that protrudes into the outflow tract of the non-systemic ventricle (morphologic left ventricle). Ultimately, our patient was felt to be too high-risk for surgical correction and a course of medical therapy has been pursued.

Keywords

Congenital heart disease Echocardiography Cardiac magnetic resonance imaging 

Supplementary material

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Supplementary material 2 (AVI 14729 kb)

Supplementary material 3 (AVI 12961 kb)

References

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    Warnes CA (2006) Transposition of the great arteries. Circulation 114:2699–2709CrossRefPubMedGoogle Scholar
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    Caso P, Ascione L, Lange A, Palka P, Mininni N, Sutherland GR (1998) Diagnostic value of transesophageal echocardiography in the assessment of congenitally corrected transposition of the great arteries in adult patients. Am Heart J 135:43–50CrossRefPubMedGoogle Scholar
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    Reddy SC, Chopra PS, Rao PS (1997) Aneurysm of the membranous ventricular septum resulting in pulmonary outflow tract obstruction in congenitally corrected transposition of the great arteries. Am Heart J 133:112–119CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, B.V. 2010

Authors and Affiliations

  1. 1.Heart & Vascular Institute, Department of Cardiovascular MedicineCleveland ClinicClevelandUSA

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