File Download
 
Links for fulltext
(May Require Subscription)
 
Supplementary

Article: Outcome of infants with right atrial isomerism: Is prognosis better with normal pulmonary venous drainage?
  • Basic View
  • Metadata View
  • XML View
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
2013 Impact Factor: 6.023
2013 SCImago Journal Rankings: 2.793
 
DOIhttp://dx.doi.org/10.1136/heart.87.2.146
 
PubMed Central IDPMC1766999
 
ISI Accession Number IDWOS:000173450000017
 
ReferencesReferences in Scopus
 
DC FieldValue
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
 
dc.format.mimetypetext/plain
 
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
2013 Impact Factor: 6.023
2013 SCImago Journal Rankings: 2.793
 
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
 
<?xml encoding="utf-8" version="1.0"?>
<item><contributor.author>Cheung, YF</contributor.author>
<contributor.author>Cheng, VYW</contributor.author>
<contributor.author>Chau, AKT</contributor.author>
<contributor.author>Chiu, CSW</contributor.author>
<contributor.author>Yung, TC</contributor.author>
<contributor.author>Leung, MP</contributor.author>
<date.accessioned>2007-01-08T02:30:47Z</date.accessioned>
<date.available>2007-01-08T02:30:47Z</date.available>
<date.issued>2002</date.issued>
<identifier.citation>Heart, 2002, v. 87 n. 2, p. 146-152</identifier.citation>
<identifier.issn>1355-6037</identifier.issn>
<identifier.uri>http://hdl.handle.net/10722/42167</identifier.uri>
<description.abstract>Objective: 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 &lt; 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.</description.abstract>
<format.extent>112936 bytes</format.extent>
<format.extent>3357 bytes</format.extent>
<format.extent>4482 bytes</format.extent>
<format.mimetype>application/pdf</format.mimetype>
<format.mimetype>text/plain</format.mimetype>
<format.mimetype>text/plain</format.mimetype>
<language>eng</language>
<publisher>B M J Publishing Group. The Journal&apos;s web site is located at http://heart.bmjjournals.com/</publisher>
<relation.ispartof>Heart</relation.ispartof>
<rights>Heart. Copyright &#169; B M J Publishing Group.</rights>
<rights>Creative Commons: Attribution 3.0 Hong Kong License</rights>
<subject.mesh>Heart atria - abnormalities - surgery</subject.mesh>
<subject.mesh>Heart bypass</subject.mesh>
<subject.mesh>Infant, newborn</subject.mesh>
<subject.mesh>Pulmonary veins - physiology</subject.mesh>
<subject.mesh>Survival analysis</subject.mesh>
<title>Outcome of infants with right atrial isomerism: Is prognosis better with normal pulmonary venous drainage?</title>
<type>Article</type>
<identifier.openurl>http://library.hku.hk:4550/resserv?sid=HKU:IR&amp;issn=1355-6037&amp;volume=87&amp;issue=2&amp;spage=146&amp;epage=152&amp;date=2002&amp;atitle=Outcome+of+infants+with+right+atrial+isomerism:+is+prognosis+better+with+normal+pulmonary+venous+drainage?</identifier.openurl>
<description.nature>published_or_final_version</description.nature>
<identifier.doi>10.1136/heart.87.2.146</identifier.doi>
<identifier.pmid>11796553</identifier.pmid>
<identifier.pmcid>PMC1766999</identifier.pmcid>
<identifier.scopus>eid_2-s2.0-0036153780</identifier.scopus>
<identifier.hkuros>65233</identifier.hkuros>
<relation.references>http://www.scopus.com/mlt/select.url?eid=2-s2.0-0036153780&amp;selection=ref&amp;src=s&amp;origin=recordpage</relation.references>
<identifier.volume>87</identifier.volume>
<identifier.issue>2</identifier.issue>
<identifier.spage>146</identifier.spage>
<identifier.epage>152</identifier.epage>
<identifier.isi>WOS:000173450000017</identifier.isi>
<publisher.place>United Kingdom</publisher.place>
<bitstream.url>http://hub.hku.hk/bitstream/10722/42167/1/65233.pdf</bitstream.url>
</item>
Author Affiliations
  1. The University of Hong Kong
  2. Grantham Hospital Hong Kong