PEDIATRICS Vol. 91 No. 2 February 1993, pp. 403-410
This Article
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Dudell, G. G.
Right arrow Articles by Lamberti, J. J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Dudell, G. G.
Right arrow Articles by Lamberti, J. J.

Common Pulmonary Vein Atresia: The Role of Extracorporeal Membrane Oxygenation

Golde G. Dudell MD1, Marva L. Evans MD1, Henry F. Krous MD2, Robert L. Spicer MD3, and John J. Lamberti MD4

1 From the Divisions of Neonatology, University of California San Diego School of Medicine
2 From the Divisions of Pathology, University of California San Diego School of Medicine
3 From the Divisions of Cardiology, University of California San Diego School of Medicine
4 From the Divisions of Cardiothoracic Surgery, Children's Hospital San Diego and University of California San Diego School of Medicine

Common pulmonary vein atresia is a rare form of cyanotic congenital heart disease in which the pulmonary veins join to form a blind confluence that does not communicate with the heart or the major systemic veins. Twenty-one cases have been reported since the lesion was first described in 1962; only two patients with this lesion have survived. Over a 4-year period, common pulmonary vein atresia was diagnosed in five newborns referred to the San Diego Regional Extracorporeal Membrane Oxygenation Program. All five improved dramatically as a result of venoarterial bypass. Congenital heart disease was diagnosed at autopsy in the initial case and by cardiac ultrasound and/or catheterization in the others. Surgical repair was attempted in three neonates; all three required continued extracorporeal membrane oxygenation support postoperatively because of pulmonary hypertension and severe pulmonary parenchymal disease. One infant died of respiratory insufficiency at 3 months of age. The other two survived and were discharged from the hospital. The diagnostic and therapeutic dilemmas posed by this lesion and the life-saving potential for extracorporeal membrane oxygenation in this rapidly fatal cardiac anomaly are the bases of this report.

Key Words: common pulmonary vein atresia • congenital heart disease • extracorporeal membrane oxygenation

Submitted on June 23, 1992
Accepted on August 20, 1992