Early neonatal death due to an unusual pattern of total anomalous pulmonary venous drainage

Total anomalous pulmonary venous drainage (TAPVD) is an uncommon congenital cardiovascular anomaly caused by failure of the common pulmonary vein to develop a connection with the common atrium in early ontogeny, resulting in persisting connections between pulmonary and systemic venous systems and a left-to-right shunt. This usually occurs via a single, enlarged, well-defined venous channel. The authors present an unusual case of TAPVD with venous obstruction with a profusely arborizing plexus of veins arising from the common pulmonary vein and forming multiple tiny anastomoses with the systemic venous system. This plexus may represent the persistence of an earlier, less involuted embryological form of the primitive pulmonary splanchnic plexus than those found in previously described patterns of TAPVD.