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REVIEW ARTICLE
Year : 2017  |  Volume : 12  |  Issue : 2  |  Page : 74-80

An overview of abnormal pulmonary venous connection in children


1 Department of Paediatrics, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria
2 Department of Paediatrics, Usman Dan Fodio University/Usman Dan Fodio University Teaching Hospital, Sokoto, Nigeria

Correspondence Address:
Mustafa O Asani
Department of Paediatrics, Bayero University/Aminu Kano Teaching Hospital, Kano
Nigeria
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DOI: 10.4103/summ.summ_2_16

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Abnormal pulmonary venous connection represents a rare variant of congenital heart disease, in which the pulmonary veins fail to make their normal connection to the left atrium. Instead, they connect and drain anomalously into the systemic venous circulation. The abnormal connection could be partial, when one or more but not all the pulmonary veins connect anomalously or total, when all the pulmonary veins connect anomalously. Total anomalous pulmonary venous connection (TAPVC) is by far the most common and most severe lesion, with a reported prevalence ranging between 1.5% and 2%. Its hemodynamics and clinical features are variable, and diagnosis can be challenging especially in resource-limited setting. This review discusses the embryology and pathogenesis of abnormal pulmonary venous connection, with emphasis on TAPVC. The clinical manifestations and various diagnostic options are also highlighted.


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