title: Heterotaxy syndrome infants are at risk for early shunt failure after Ladd procedure.
abstract: BACKGROUND
  Cardiac-specific risks and complications after a Ladd procedure in patients with heterotaxy syndrome (HS) and intestinal rotational anomalies (IRA) are unknown. We sought to (1) describe rates of hospital mortality and early systemic-to-pulmonary (S-P) artery shunt failure after the Ladd procedure in patients with HS and (2) explore risk factors associated with early shunt failure in patients with HS with single ventricle (SV).
  
  
  METHODS
  This retrospective study included all Ladd procedures performed from January 1999 to December 2012 in patients with HS at a single center. Risk factors investigated for early S-P artery shunt failure included birth weight, gestational age, sex, age at and timing of Ladd procedure relative to cardiac operations, and shunt type.
  
  
  RESULTS
  Ladd procedure was performed on 54 infants with HS and congenital heart disease. Hospital mortality for the entire cohort was 5.6% (3 of 54 patients). Early shunt failure occurred in 19% (4 of 21) of HS infants with SV. Mean preoperative blood urea nitrogen (BUN) levels were higher in HS infants with early shunt failure (20 versus 12.5 mg/dL; p = 0.054).
  
  
  CONCLUSIONS
  Patients with SV and HS with S-P artery shunts are at risk for early shunt failure after a Ladd procedure. A higher mean preoperative BUN level is noted in patients with HS and early shunt failure. Careful risk-benefit analysis is indicated before recommending routine elective Ladd procedures in patients with HS.
authors.affiliation: Department of Pediatrics, Columbia University Medical Center, New York, New York.
authors.forename: Shawndip
authors.initials: S
authors.lastname: Sen
authors.affiliation: Department of Pediatrics, Columbia University Medical Center, New York, New York.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Duchon
authors.affiliation: Department of Radiology, Columbia University Medical Center, New York, New York.
authors.forename: Brooke
authors.initials: B
authors.lastname: Lampl
authors.affiliation: Department of Surgery, Columbia University Medical Center, New York, New York.
authors.forename: Gudrun
authors.initials: G
authors.lastname: Aspelund
authors.affiliation: Department of Surgery, Columbia University Medical Center, New York, New York.
authors.forename: Emile
authors.initials: E
authors.lastname: Bacha
authors.affiliation: Department of Pediatrics, Columbia University Medical Center, New York, New York. Electronic address: gk2008@columbia.edu.
authors.forename: Ganga
authors.initials: G
authors.lastname: Krishnamurthy
mesh_terms: D000714:Anastomosis, Surgical; D004065:Digestive System Abnormalities; D013505:Digestive System Surgical Procedures / Q000379:methods; D005260:Female; D059446:Heterotaxy Syndrome / Q000150:complications / Q000601:surgery*; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D045822:Intestinal Volvulus / Q000150:complications / Q000151:congenital* / Q000601:surgery; D008297:Male; D011651:Pulmonary Artery / Q000601:surgery*; D012189:Retrospective Studies; D012307:Risk Factors; D017211:Treatment Failure