Common pulmonary vein atresia: report of three cases and review of the literature

Abstract

Common pulmonary vein atresia is a rare and usually fatal congenital anomaly, in which the pulmonary veins come together to form a confluence that does not connect to the left atrium. We report our experience with three cases of common pulmonary vein atresia and review the literature on this anomaly. The diagnosis of common pulmonary vein atresia must be entertained in any newborn that presents with cyanosis, refractory acidosis, and decreased systemic perfusion within the first 48 hours of life. Echocardiography is a useful screening tool, but cardiac catheterisation is the preferred diagnostic tool. Common pulmonary vein atresia can be fatal without surgical intervention, but survival after surgery continues to be poor.

Comparison of prenatal ultrasound and postmortem findings in fetuses with common pulmonary vein atresia

“…This discrepancy can be deemed as one of the distinguishing points between the two. 18 However, our results showed no significant difference in the incidence of complex cardiac malformations and AS between the CPVA and TAPVC groups. Therefore, the characteristics of coexisting malformations in TAPVR and CPVA cases may provide little help in the diagnosis or differential diagnosis of these conditions.…”

“…It is reported that TAPVC is liable to be accompanied by complicated heart malformations or AS, while CPVA seldom coexists with other complex malformations. This discrepancy can be deemed as one of the distinguishing points between the two . However, our results showed no significant difference in the incidence of complex cardiac malformations and AS between the CPVA and TAPVC groups.…”

“…The previous reports on CPVA were mainly confined to neonatal cases, and there was a lack of fetal cases. 2,3,18 In this study, the 6 fetuses had other associated complex cardiac malformations and AS. Therefore, those fetuses were likely to die even at birth, and there was no time for further treatment or definitive diagnosis.…”

“…Our study compared 6 cases of fetal CPVA with 31 neonatal cases reported by Perez . The results showed that the incidence of coexisting cardiac anomalies or AS in the fetal group was significantly higher than that in neonatal group.…”

“…The reason was, on one hand, related to differing patient selection. The previous reports on CPVA were mainly confined to neonatal cases, and there was a lack of fetal cases . In this study, the 6 fetuses had other associated complex cardiac malformations and AS.…”