Obstructive total anomalous pulmonary venous drainage.

ABSTRACT

Obstructive total anomalous pulmonary venous drainage (TAPVD) has a florid presentation in the neonatal period from the resulting pulmonary edema. A complete anatomical and functional diagnosis is usually possible by transthoracic color Doppler echocardiography, and cardiac catheterization with its attendant risks can usually be avoided in the sick neonate. Currently balloon atrial septostomy has a limited role in palliation of these neonates. Corrective surgery on urgent basis has gratifying results and prevents progression of pulmonary vascular occlusive disease. Pulmonary hypertensive crisis in the early post-operative course needs to be avoided and treated vigorously if it were to develop. Late post-operative course can be complicated by anastomotic obstruction or progressive narrowing of the individual pulmonary veins. Although the former can be dealt successfully by re-operation, surgical treatment of the latter is not satisfactory. Balloon dilatation of the obstructed pulmonary venous pathways, native and post-operative, has been reported with equivocal results. Infant heart-lung transplant is a viable option in patients with diffusely narrow pulmonary veins or complex TAPVDs.