Congenitally corrected transposition with isolated atrial septal defect and apicocaval juxtaposition: a hitherto undescribed cardiac cause of central cyanosis

  • Neeraj AwasthyEmail author
  • Rajesh Sharma
Case Report


In our practice, we have been struck by the presence of central hypoxemia in individuals with congenitally corrected transposition of the great arteries (CCTGA) with an atrial septal defect (ASD) as the only association, despite existence of normal pulmonary artery pressures. This right to left shunting at the atrial level occurs only in those patients where there is additional presence of apicocaval juxtaposition (ACJ). Patients with CCTGA but without ACJ manifest only left to right shunting at the level of the ASD. We present a case series of patients with CCTGA with intact ventricular septum and incomplete atrial septum with a possible basis for this phenomenon.


Cyanosis Corrected TGA Av-va discordance 


Compliance with ethical standards

We declare that there has been compliance with the ethical standards as per the journal protocols and informed consent was obtained from all participants.

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Supplementary material

Movie 1

Contrast study showing contrast injected in the left antecubital vein filling the left and the right ventricles in a patient with CCTGA, ASD, and ACJ. (AVI 29900 kb)


  1. 1.
    Awasthy N, Ambatkar P, Radhakrishnan S, Iyer KS. Lutembacher syndrome with unroofed left superior vena cava: a diagnostic dilemma. Pediatric Cardiol. 2013;34:1985–8.CrossRefGoogle Scholar
  2. 2.
    Webb CL. Congenitally corrected transposition of the great arteries: clinical features, diagnosis, and prognosis. Prog Ped Cardiol. 1999;10:17–30.CrossRefGoogle Scholar
  3. 3.
    Beauchesne LM, Warnes CA, Connolly HM, Ammash NM, Tajik AJ, Danielson GK. Outcome of the unoperated adult who presents with congenitally corrected transposition of the great arteries. J Am Coll Cardiol. 2002;40:285–90.CrossRefPubMedGoogle Scholar

Copyright information

© Indian Association of Cardiovascular-Thoracic Surgeons 2019

Authors and Affiliations

  1. 1.Department of Pediatric CardiologyMax HospitalDelhiIndia
  2. 2.Department of Pediatric Cardiac SurgeryJaypee Hospital UPNoidaIndia

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