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

TitleOutcome of infants with right atrial isomerism: Is prognosis better with normal pulmonary venous drainage?
Authors
Issue Date2002
PublisherB M J Publishing Group. The Journal's web site is located at http://heart.bmjjournals.com/
Citation
Heart, 2002, v. 87 n. 2, p. 146-152 How to Cite?
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 < 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.
Persistent Identifierhttp://hdl.handle.net/10722/42167
ISSN
2013 Impact Factor: 6.023
2013 SCImago Journal Rankings: 2.793
PubMed Central ID
ISI Accession Number ID
References

 

Author Affiliations
  1. The University of Hong Kong
  2. Grantham Hospital Hong Kong
DC FieldValueLanguage
dc.contributor.authorCheung, YFen_HK
dc.contributor.authorCheng, VYWen_HK
dc.contributor.authorChau, AKTen_HK
dc.contributor.authorChiu, CSWen_HK
dc.contributor.authorYung, TCen_HK
dc.contributor.authorLeung, MPen_HK
dc.date.accessioned2007-01-08T02:30:47Z-
dc.date.available2007-01-08T02:30:47Z-
dc.date.issued2002en_HK
dc.identifier.citationHeart, 2002, v. 87 n. 2, p. 146-152en_HK
dc.identifier.issn1355-6037en_HK
dc.identifier.urihttp://hdl.handle.net/10722/42167-
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.en_HK
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.languageengen_HK
dc.publisherB M J Publishing Group. The Journal's web site is located at http://heart.bmjjournals.com/en_HK
dc.relation.ispartofHearten_HK
dc.rightsHeart. Copyright © B M J Publishing Group.en_HK
dc.rightsCreative Commons: Attribution 3.0 Hong Kong License-
dc.subject.meshHeart atria - abnormalities - surgeryen_HK
dc.subject.meshHeart bypassen_HK
dc.subject.meshInfant, newbornen_HK
dc.subject.meshPulmonary veins - physiologyen_HK
dc.subject.meshSurvival analysisen_HK
dc.titleOutcome of infants with right atrial isomerism: Is prognosis better with normal pulmonary venous drainage?en_HK
dc.typeArticleen_HK
dc.identifier.openurlhttp://library.hku.hk:4550/resserv?sid=HKU:IR&issn=1355-6037&volume=87&issue=2&spage=146&epage=152&date=2002&atitle=Outcome+of+infants+with+right+atrial+isomerism:+is+prognosis+better+with+normal+pulmonary+venous+drainage?en_HK
dc.identifier.emailCheung, YF:xfcheung@hku.hken_HK
dc.identifier.authorityCheung, YF=rp00382en_HK
dc.description.naturepublished_or_final_versionen_HK
dc.identifier.doi10.1136/heart.87.2.146en_HK
dc.identifier.pmid11796553en_HK
dc.identifier.pmcidPMC1766999-
dc.identifier.scopuseid_2-s2.0-0036153780en_HK
dc.identifier.hkuros65233-
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-0036153780&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume87en_HK
dc.identifier.issue2en_HK
dc.identifier.spage146en_HK
dc.identifier.epage152en_HK
dc.identifier.isiWOS:000173450000017-
dc.publisher.placeUnited Kingdomen_HK
dc.identifier.scopusauthoridCheung, YF=7202111067en_HK
dc.identifier.scopusauthoridCheng, VYW=7005529517en_HK
dc.identifier.scopusauthoridChau, AKT=35787094400en_HK
dc.identifier.scopusauthoridChiu, CSW=8714554800en_HK
dc.identifier.scopusauthoridYung, TC=9132842300en_HK
dc.identifier.scopusauthoridLeung, MP=7201944800en_HK

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