Document Detail

Rare variant of total anomalous pulmonary venous connection: intrapulmonary drainage of one lung by the other--a case report and review of the literature.
MedLine Citation:
PMID:  9050067     Owner:  NLM     Status:  MEDLINE    
Total anomalous pulmonary venous connection is an uncommon congenital cardiovascular anomaly but one that occurs frequently in the asplenia syndrome. We present a rare type of total anomalous pulmonary venous connection in a 4-month-old baby with the asplenia syndrome. In this patient, all of the left pulmonary veins united to form a common pulmonary vein, which crossed the mediastinum and then proceeded in an intrapulmonary fashion in the right lung. A small pulmonary vein emerged from the right upper lobe and drained into the right superior vena cava. Nine other cases have been reported, four also with asplenia, five with otherwise normal cardiac anatomy apart from an atrial septal defect. The incidence of pulmonary venous obstruction is high, at least 70%. Two cases, both with a normal heart, had corrective surgery. One with no pulmonary venous obstruction was repaired successfully. Embryologically, this type of total anomalous pulmonary venous connection represents a developmental error early in embryonic development before the venous connection between the two lungs and the cardinal veins involute.
J H Chung; Y L Suh; H J Lee; I S Kang; Y H Choe; H J Ree
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association     Volume:  17     ISSN:  1077-1042     ISO Abbreviation:  Pediatr Pathol Lab Med     Publication Date:    1997 Jan-Feb
Date Detail:
Created Date:  1997-05-12     Completed Date:  1997-05-12     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  9518033     Medline TA:  Pediatr Pathol Lab Med     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  133-40     Citation Subset:  IM    
Department of Pathology, Seoul National University College of Medicine, Korea.
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MeSH Terms
Genetic Variation*
Pulmonary Circulation*
Pulmonary Veins / abnormalities*,  pathology

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