title: Interstage mortality for functional single ventricle with heterotaxy syndrome: a retrospective study of the clinical experience of a single tertiary center.
abstract: BACKGROUND
  In spite of improved survival after palliation for single ventricle, interstage mortality for a single ventricle with heterotaxy syndrome is unknown. The purpose of this study was to quantify interstage mortality and influence mortality risk factors.
  
  
  METHODS
  From November 1994 until February 2012, all patients that had a functional single ventricle and heterotaxy syndrome who underwent palliative operations at our center were included. Patients with hypoplastic left heart syndrome and operative mortality cases were excluded. The factors that influenced interstage mortality were determined by multivariate Cox analysis.
  
  
  RESULTS
  There were 16 patients with interstage mortality (41.0%), much higher than the non-heterotaxy group (vs. 11.3%, P = 0.001, OR = 5.478). The major presumptive causes of death were infection or sepsis (37.5%) and unknown sudden death (31.3%). When we compared the survival group and the mortality group with heterotaxy syndrome, Blalock-Taussig shunt as a 1st palliation is most common for both groups but there were more for the mortality group (81.2% vs. 52.2%), and there were more with bidirectional cavo-pulmonary shunt as a 1st palliation in the survival group (10 patients vs. 2 patients). The existence of pulmonary vein stenosis at initial diagnosis was more common for the mortality group. In multivariate Cox analysis, however, the duration of hospitalization at palliation, the duration of intensive care unit stay after palliation and the existence of pulmonary vein stenosis at diagnosis were significant risk factors.
  
  
  CONCLUSIONS
  Interstage mortality for a functional single ventricle with heterotaxy syndrome is significantly higher than for non-heterotaxy syndrome. Therefore more attention should be given to the prevention of interstage mortality in these patients with risk factors.
authors.affiliation: Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Irwon-dong, Gangnam-gu, Seoul 135-710, South Korea.
authors.forename: Jinyoung
authors.initials: J
authors.lastname: Song
authors.forename: I-Seok
authors.initials: IS
authors.lastname: Kang
authors.forename: June
authors.initials: J
authors.lastname: Huh
authors.forename: Ok Jeong
authors.initials: OJ
authors.lastname: Lee
authors.forename: Geena
authors.initials: G
authors.lastname: Kim
authors.forename: Tae Gook
authors.initials: TG
authors.lastname: Jun
authors.forename: Ji Hyuk
authors.initials: JH
authors.lastname: Yang
mesh_terms: D000293:Adolescent; D058366:Blalock-Taussig Procedure; D002648:Child; D002675:Child, Preschool; D005260:Female; D006352:Heart Ventricles / Q000002:abnormalities* / Q000601:surgery; D059446:Heterotaxy Syndrome / Q000401:mortality* / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D015999:Multivariate Analysis; D012189:Retrospective Studies; D012307:Risk Factors; D062606:Tertiary Care Centers; D055815:Young Adult