Totally anomalous pulmonary venous connection with intact atrial septum.

Background: Total anomalous pulmonary venous connection with intact interatrial septum is extremely rare.
Aims: In these patients, a right to left shunt through a ventricular septal defect or a patent ductus arteriosus is mandatory to maintain the systemic circulation. Mechanical or physiological restriction of shunting through these pathways results in rapid clinical deterioration.
Discussion: We describe the anatomical findings with surgical repair in one such 10-day-old baby.
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