Document Detail

Surgical repair of total anomalous pulmonary venous connection.
MedLine Citation:
PMID:  16638546     Owner:  NLM     Status:  MEDLINE    
The diagnosis of total anomalous pulmonary venous connection (TAPVC) is made when all four pulmonary veins drain anomalously to the right atrium or to a tributary of the systemic veins. It constitutes between 1% and 1.5% of all children with congenital heart disease and can be categorized by the site of drainage into the systemic circulation (supracardiac, 45%; infracardiac, 25%; cardiac, 25%; mixed, 5%). The clinical presentation is different if the pulmonary venous drainage is unobstructed (heart failure, mild cyanosis) or obstructed (respiratory failure, severe heart failure). Surgical management depends on the anatomic type. Obstructed TAPVC requires urgent surgical intervention, whereas unobstructed TAPVC can be dealt with electively; although this is usually operated on once the diagnosis is made. Postoperative pulmonary artery hypertension can be problematic. Recent surgical results with isolated TAPVC have improved, with operative mortality consistently at less than 10%. A particularly challenging group of patients are those with single ventricle physiology and TAPVC with high operative mortality and poor long-term survival.
Kirk R Kanter
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual     Volume:  -     ISSN:  1092-9126     ISO Abbreviation:  Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu     Publication Date:  2006  
Date Detail:
Created Date:  2006-04-26     Completed Date:  2006-06-29     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9815944     Medline TA:  Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu     Country:  United States    
Other Details:
Languages:  eng     Pagination:  40-4     Citation Subset:  IM    
Division of Cardio-Thoracic Surgery, Department of Surgery, Emory University School of Medicine and Children's Healthcare of Atlanta at Egleston, Atlanta, GA 30322, USA.
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MeSH Terms
Anastomosis, Surgical / adverse effects
Cardiac Surgical Procedures / adverse effects,  methods*
Circulatory Arrest, Deep Hypothermia Induced
Heart Atria / surgery*
Heart Defects, Congenital / surgery*
Hypertension, Pulmonary / etiology,  therapy
Pulmonary Veins / abnormalities*,  surgery*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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