Common pulmonary vein atresia: the role of extracorporeal membrane oxygenation.
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.
Dudell
G G
GG
Division of Neonatology, Children's Hospital, San Diego, CA 92123.
Evans
M L
ML
Krous
H F
HF
Spicer
R L
RL
Lamberti
J J
JJ
D001034:Apgar Score; D001784:Blood Gas Analysis; D002140:California / Q000453:epidemiology; D006328:Cardiac Catheterization; D006348:Cardiac Surgical Procedures; D000013:Congenital Abnormalities / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D003937:Diagnosis, Differential; D004452:Echocardiography; D015199:Extracorporeal Membrane Oxygenation / Q000379:methods / Q000592:standards*; D005260:Female; D005500:Follow-Up Studies; D006776:Hospitals, Pediatric; D006785:Hospitals, University; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011667:Pulmonary Veins / Q000002:abnormalities*; D012017:Referral and Consultation; D015996:Survival Rate; D016896:Treatment Outcome