Atresia of the common pulmonary vein--a rare congenital anomaly.
OBJECTIVE
Early atresia of the common pulmonary vein (ACPV) leads to total anomalous pulmonary venous drainage, while late atresia or incomplete absorption leads to common pulmonary vein atresia and cor triatriatum sinister (both of which are rare). We report seven cases of atresia of the common pulmonary vein at autopsy.


METHODS
Retrospective case records studied.


METHODS
Tertiary care teaching hospital affiliated to medical college in Mumbai, India.


METHODS
The clinical and autopsy records of neonates and infants diagnosed with ACPV over a period of 11 years were reviewed. The demographic data, clinical features, and results of investigations were correlated with the cardiac findings at necropsy.


RESULTS
Seven neonates and infants (five males and two females) had ACPV. Six babies presented at birth and expired within 48 hours. They had a homogeneous group of symptoms of cyanosis since birth with respiratory distress and/or features of congestive cardiac failure. One had perimembranous ventricular septal defect with bicuspid pulmonary valve and atresia of aortic valve. Two had dysmorphic facial features suggestive of Down's syndrome. Isolated ACPV was seen in only two patients. Asplenia syndrome was seen in three patients. Marked dilatation of the pulmonary lymphatics was identified in three patients.


CONCLUSIONS
Early atresia of the common pulmonary vein, an extremely rare abnormality, manifests in early infancy/neonatal period and needs urgent corrective surgery. It is associated with other congenital heart disorders and extracardiac manifestations as well.
Vaideeswar
Pradeep
P
Department of Pathology, Cardiovascular & Thoracic Division, Seth G.S. Medical College, KEM Hospital, Parel, Mumbai, India. shreeprajai@yahoo.co.in
Tullu
Milind S
MS
Sathe
Pragati A
PA
Nanavati
Ruchi
R
D000015:Abnormalities, Multiple / Q000473:pathology; D001344:Autopsy; D003490:Cyanosis / Q000209:etiology; D004314:Down Syndrome / Q000150:complications; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications; D006333:Heart Failure / Q000209:etiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000473:pathology; D012127:Respiratory Distress Syndrome, Newborn / Q000209:etiology; D012189:Retrospective Studies; D013154:Spleen / Q000002:abnormalities; D054079:Vascular Malformations / Q000150:complications* / Q000473:pathology