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