Atresia of the bilateral pulmonary veins: a rare and dismal anomaly identified on cardiac CT.

BACKGROUND

Imaging findings of bilateral pulmonary vein atresia have not been described.

OBJECTIVE

To describe cardiac CT findings and clinical outcomes of bilateral pulmonary vein atresia.

MATERIALS AND

METHODS

Three newborns with bilateral pulmonary vein atresia were encountered at our institution during a period of 8 years. We evaluated prenatal echocardiographic findings, clinical presentations, postnatal echocardiographic findings, chest radiographic findings, cardiac CT findings and clinical outcomes.

RESULTS

All newborns presented immediately after birth with severe cyanosis, respiratory distress and acidosis that were unresponsive to medical management. Prenatal and postnatal echocardiographic studies and chest radiography were misleading, inconclusive or nonspecific in making the diagnosis in these children; however cardiac CT clearly demonstrated atresia of the bilateral pulmonary veins with multiple small mediastinal collateral veins and pulmonary edema. Surgical treatments were not feasible for this anomaly. Their clinical outcomes were universally dismal and all infants died within 3 days.

CONCLUSION

Cardiac CT provides an accurate diagnosis of bilateral pulmonary vein atresia and leads to prompt treatment decision in these children.