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Case Report: Late Presentation of Congenitally Corrected Transposition of Great Arteries with Large Ventricular Septal Defect

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Congenitally corrected transposition of great arteries (ccTGA) is a rare heart defect. Untreated ccTGA with large ventricular septal defect (VSD) is often fatal.

In this case report, the authors report a late presentation ccTGA associated with large VSD in the absence of severe pulmonary hypertension or systemic ventricle dysfunction. The authors determined that severe pulmonary valve stenosis helped maintain a stable condition until the patient was in the fourth decade of life.

Multimodality imaging including transthoracic echocardiogram, transesophageal echography, cardiac magnetic resonance imaging, and cardiac computed tomography were used for diagnosis.

The authors concluded that pulmonary stenosis can delay the need for surgical correction of large VSD in ccTGA patients, although the decision should be made on an individual basis.


Elenizi K, Alharthi R, Matta A, et al. Late presentation of a congenitally corrected transposition of great arteries and hemodynamically balanced ventricles associated with a large ventricular septal defect and severe pulmonary stenosis: a case report and a multi-imaging approach. Am J Case Rep. 2020;21:e920822.

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