Article: Outcome of infants with right atrial isomerism: Is prognosis better with normal pulmonary venous drainage?

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TitleOutcome of infants with right atrial isomerism: Is prognosis better with normal pulmonary venous drainage?
AuthorsCheung, YF1
Cheng, VYW1
Chau, AKT1
Chiu, CSW2
Yung, TC1
Leung, MP1
Issue Date2002
PublisherB M J Publishing Group. The Journal's web site is located at http://heart.bmjjournals.com/
CitationHeart, 2002, v. 87 n. 2, p. 146-152 [How to Cite?]
DOI: http://dx.doi.org/10.1136/heart.87.2.146
AbstractObjective: To compare the outcome of infants and children who have right atrial isomerism and normal pulmonary venous drainage with those who have anomalous drainage, and to determine factors associated with poor outcome. Design and patients: Retrospective review of management and outcome of 116 infants and children determined to have right atrial isomerism between January 1980 and December 2000. Setting: Tertiary paediatric cardiac centre. Results: The 116 patients presented at a median of one day (range 1 day to 3.7 years) with cyanosis in the majority (96%). No interventions were planned in 31 (27%) patients, all of whom died. The early surgical mortality for pulmonary venous repair was 25% (2 of 8), Fontan procedure 26% (5 of 19), cavopulmonary shunting 7.7% (1 of 13), and systemic pulmonary arterial shunt insertion 1.9% (1 of 53). Late mortality was related to infection (n = 10), sudden death of unknown aetiology (n = 7, 5 with history of arrhythmia), and documented arrhythmia (n = 1). Patients with obstructed anomalous pulmonary venous drainage had the worst survival (p < 0.001). The mean (SEM) survival estimates for those with normal pulmonary venous drainage at 1, 5, 10, and 15 years was 81 (5.3)%, 67 (6.6)%, 60 (7.8)%, and 43 (12)%, respectively, similar to those for patients with non-obstructed anomalous drainage (p = 0.06). Independent risk factors for mortality included pulmonary venous obstruction (relative risk 3.8, p = 0.001) and a single ventricle (relative risk 2.9, p = 0.016). An analysis of only patients with normal pulmonary venous drainage identified no risk factors for mortality. Conclusions: The long term outcome of infants and children with right atrial isomerism in association with a normal pulmonary venous drainage remains unfavourable. Sepsis and sudden death that may potentially be related to cardiac arrhythmia are major causes of late mortality.
ISSN1355-6037
2011 Impact Factor: 4.223
2011 SCImago Journal Rankings: 0.348
DOIhttp://dx.doi.org/10.1136/heart.87.2.146
PubMed Central IDPMC1766999
ReferencesReferences in Scopus
DC Field
Value
dc.contributor.authorCheung, YF
dc.contributor.authorCheng, VYW
dc.contributor.authorChau, AKT
dc.contributor.authorChiu, CSW
dc.contributor.authorYung, TC
dc.contributor.authorLeung, MP
dc.date.accessioned2007-01-08T02:30:47Z
dc.date.available2007-01-08T02:30:47Z
dc.date.issued2002
dc.description.abstractObjective: To compare the outcome of infants and children who have right atrial isomerism and normal pulmonary venous drainage with those who have anomalous drainage, and to determine factors associated with poor outcome. Design and patients: Retrospective review of management and outcome of 116 infants and children determined to have right atrial isomerism between January 1980 and December 2000. Setting: Tertiary paediatric cardiac centre. Results: The 116 patients presented at a median of one day (range 1 day to 3.7 years) with cyanosis in the majority (96%). No interventions were planned in 31 (27%) patients, all of whom died. The early surgical mortality for pulmonary venous repair was 25% (2 of 8), Fontan procedure 26% (5 of 19), cavopulmonary shunting 7.7% (1 of 13), and systemic pulmonary arterial shunt insertion 1.9% (1 of 53). Late mortality was related to infection (n = 10), sudden death of unknown aetiology (n = 7, 5 with history of arrhythmia), and documented arrhythmia (n = 1). Patients with obstructed anomalous pulmonary venous drainage had the worst survival (p < 0.001). The mean (SEM) survival estimates for those with normal pulmonary venous drainage at 1, 5, 10, and 15 years was 81 (5.3)%, 67 (6.6)%, 60 (7.8)%, and 43 (12)%, respectively, similar to those for patients with non-obstructed anomalous drainage (p = 0.06). Independent risk factors for mortality included pulmonary venous obstruction (relative risk 3.8, p = 0.001) and a single ventricle (relative risk 2.9, p = 0.016). An analysis of only patients with normal pulmonary venous drainage identified no risk factors for mortality. Conclusions: The long term outcome of infants and children with right atrial isomerism in association with a normal pulmonary venous drainage remains unfavourable. Sepsis and sudden death that may potentially be related to cardiac arrhythmia are major causes of late mortality.
dc.description.naturepublished_or_final_version
dc.format.extent112936 bytes
dc.format.extent3357 bytes
dc.format.extent4482 bytes
dc.format.mimetypeapplication/pdf
dc.format.mimetypetext/plain
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dc.identifier.citationHeart, 2002, v. 87 n. 2, p. 146-152 [How to Cite?]
DOI: http://dx.doi.org/10.1136/heart.87.2.146
dc.identifier.doihttp://dx.doi.org/10.1136/heart.87.2.146
dc.identifier.epage152
dc.identifier.hkuros65233
dc.identifier.isiWOS:000173450000017
dc.identifier.issn1355-6037
2011 Impact Factor: 4.223
2011 SCImago Journal Rankings: 0.348
dc.identifier.issue2
dc.identifier.openurl
dc.identifier.pmcidPMC1766999
dc.identifier.pmid11796553
dc.identifier.scopuseid_2-s2.0-0036153780
dc.identifier.spage146
dc.identifier.urihttp://hdl.handle.net/10722/42167
dc.identifier.volume87
dc.languageeng
dc.publisherB M J Publishing Group. The Journal's web site is located at http://heart.bmjjournals.com/
dc.publisher.placeUnited Kingdom
dc.relation.ispartofHeart
dc.relation.referencesReferences in Scopus
dc.rightsHeart. Copyright © B M J Publishing Group.
dc.rightsCreative Commons: Attribution 3.0 Hong Kong License
dc.subject.meshHeart atria - abnormalities - surgery
dc.subject.meshHeart bypass
dc.subject.meshInfant, newborn
dc.subject.meshPulmonary veins - physiology
dc.subject.meshSurvival analysis
dc.titleOutcome of infants with right atrial isomerism: Is prognosis better with normal pulmonary venous drainage?
dc.typeArticle
Author Affiliations
  1. The University of Hong Kong
  2. Grantham Hospital Hong Kong