Surgical correction of congenitally corrected TGA

Surgical correction of congenitally corrected TGA (transposition of great arteries)

Surgical correction of congenitally corrected TGA: Congenitally corrected transposition of great arteries (c-TGA) is also known as l-transposition of great arteries (l-TGA). Definitive correction of c-TGA is known as double switch. Conventional arterial switch (single switch) or Jatene’s procedure is the treatment of choice for d-TGA in new born period.

Even though it might appear that physiology of independent series pulmonary and systemic circulations is conceptually proper, the prognosis of patients with c-TGA is not that good, even in the absence of other associated cardiac defects [1]. Hence the need for surgical correction.

The morphological right ventricle fails in one third of cases in those without associated lesions by the fifth decade of life while it occurs in two thirds who had prior surgery for associated lesions, by 45 years of age. This is despite the fact that a nonagenerian has presented with c-TGA [2].

The systemic right ventricle supplied by a single right coronary artery has myocardial oxygen supply/demand mismatch which can lead to subendocardial ischemia, progressive fibrosis and overt heart failure later. More over Ebstein like malformation is common and causes regurgitation of the systemic atrioventricular valve in c-TGA.

Reference

1. Catherine Deshaies, Paul Khairy. The Paradox of Choice in the Surgical Management of Congenitally Corrected Transposition: What Should We Do With All of These Options Supported by Little Evidence? Transl Pediatr. 2018 Jan;7(1):5-8.
2. Elizabeth A Orchard, Oliver Ormerod, Saul Myerson, Stephen Westaby. Congenitally Corrected Transposition of the Great Arteries Presenting in a Nonagenarian. Circulation. 2010 Aug 31;122(9):e441-4.