Anomalous pulmonary venous return

Anomalous pulmonary venous return refers to a form of rare cyanotic congenital heart disease characterised by som or all of the pulmonary venous return entering the systemic circulation through embryological connections.

In case of total anomalous pulmonary venous return (TAPVR), the entire pulmonary venous return enters the systemic circulation, while in case of partial anomalous pulmonary venous return (PAPVR), only some of the pulmonary venous return enters the systemic circulation.

The patient may be anywhere from mildly symptomatic to having severe heart failure. Echocardiography makes the diagnosis. Surgical repair is required.

Diagnosis and evaluation

In case of TAPVR, chest x-ray may show the snowman sign, as the heart makes a snowman-shape.

In case of PAPVR, chest x-ray may show Scimitar syndrome, as the anomalous draining vein makes a scimitar-shape. ‎