Atresia of right pulmonary veins and anomalous left pulmonary venous drainage into portal circulation

Abstract

Thorax, 29, 446-450. Atresia of right pulmonary veins and anomalous left pulmonary venous drainage into portal circulation. An anomaly of pulmonary venous drainage in a male newborn infant is described whereby the left pulmonary veins entered the portal vein and the right pulmonary veins were atretic. A correct diagnosis was made by detecting high-oxygen saturation in the hepatic veins, right-to-left shunt at atrial level, and increased pulmonary artery wedge pressure in comparison with the left atrial pressure, and was confirmed by angiography.Atresia of one pulmonary vein or of the common pulmonary vein is an extremely rare condition. Postmortem findings in two cases of pulmonary venous atresia were described by Benesova (1955). Pulmonary veins converged to form a trunk which terminated as a capillary network in the posterior mediastinum, obviously representing a collateral route. Lucas et al. (1962) reported three similar cases of pulmonary venous atresia diagnosed at necropsy.

Supracardiac anomalous pulmonary venous connection with unilateral pulmonary venous atresia: Diagnosis and management

“…In a similar single reported case of infradiaphragmatic pulmonary venous connection and unilateral pulmonary venous atresia, the authors did not operate on their patient and mentioned unfavorable prognosis of such an entity. [ 9 ]…”

Atresia of a solitary pulmonary vein

“…5 Cardiac catheterization can then demonstrate pulmonary hypertension and raised pulmonary wedge pressures. 19 ' 20 Pulmonary arteriography can show erroneously a small pulmonary artery with no visible pulmonary veins during the levophase, a compensatory dilation of bronchial veins and lymphatic vessels on the affected side, and a preferential flow to the normal side. 16 ' 21 By means of pulmonary wedge angiography, filling of small (atretic) pulmonary veins anastomosed to small bronchial veins, as well as a venous bronchial network communicating with the azygos system can be seen.…”