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Transposition of the great arteries

Squarcia, Umberto; Macchi, Chiara

doi: 10.1097/MOP.0b013e32834aa551
Cardiovascular medicine: Edited by James C. Huhta

Purpose of review Transposition of the great arteries (TGA) is a complex congenital heart defect usually defined within the group of conotruncal defects. Some astonishing similarities between the spiral pattern of great arteries and the spiral pattern of snail shells and a possible common genetic mechanism of normal and abnormal anatomical aspects of the heart and shells are examined.

Recent findings The pulmonary vascular resistances in TGA and ventricular septal defect (VSD) need to be assessed before surgery, as they are the key factors for the success of the surgical procedure. A noninvasive method has been proposed to assess this key factor. A first series of the pregnancy outcomes in young women after arterial switch operation (ASO) is promising and encouraging for even better results. The systemic failing right ventricle (RV) is treated empirically using the same drugs and devices as for the failing left ventricle. The rationale for the treatment of ventricular failure, similar or different for predominantly right or left ventricle, is debated. The results of Rastelli operation are compared with those of the other surgical procedures for the treatment of TGA, VSD and pulmonary stenosis, namely reparation a l’ètage ventriculaire and Nikaidoh interventions.

Summary This review outlines some new aspects of the embryologic cardiac development and reveals astonishing similarities between heart and shells. A new diagnostic noninvasive method for measuring pulmonary vascular resistances, the pregnancy outcome of a first series of women operated by ASO, and the pharmacological and cardiac devices used in the failing systemic RV are presented. Finally, the review comments on the Rastelli operation as the ‘gold standard’ for TGA, VSD, and pulmonary stenosis.

Dipartimento dell’età evolutiva, Cattedra di Cardiologia Pediatrica, Università degli Studi di Parma, Parma, Italy

Correspondence to Umberto Squarcia, MD, FACC Professor of Pediatric Cardiology Università di Parma, via Gramsci 14, 43100 Parma, ItalyTel: +39 0521 1711030; e-mail:

© 2011 Lippincott Williams & Wilkins, Inc.