Atresia of the common pulmonary vein

In our study of 1326 autopsied cases of congenital heart disease, we have encountered 3 cases of atresia of the common pulmonary vein. This is a rare condition of which, to the best part of our knowledge, only 19 cases have been reported so far. Interestingly, two of our cases were associated with isomerism of the right atrial appendages, and one also had a common arterial trunk. The association with isomerism of the right appendages, and with absence of the spleen, has been observed previously but no previous case of which we are aware has had a common arterial trunk. Atresia of the common pulmonary vein is part of the spectrum related to totally anomalous pulmonary venous connexion, a lesion known to be frequently associated with isomerism of the right atrial appendages. Atresia of the common pulmonary vein, however, has not commonly been found with isomerism of the right appendages.