title: Increased postoperative respiratory complications in heterotaxy congenital heart disease patients with respiratory ciliary dysfunction.
abstract: OBJECTIVE
  Congenital heart disease (CHD) and heterotaxy patients have increased postoperative and respiratory complications. We recently showed CHD-heterotaxy patients can have respiratory ciliary dysfunction (CD) similar to that associated with primary ciliary dyskinesia, including low nasal nitric oxide and abnormal ciliary motion. In this study, we investigated whether CHD-heterotaxy patients with CD may have worse postsurgical outcomes.
  
  
  METHODS
  We examined postsurgical outcome in 13 heterotaxy-CHD patients with CD (25 surgeries), compared with 14 heterotaxy-CHD patients without CD (27 surgeries). Outcome data were collected for each surgery, including respiratory complications, tracheostomy, use of inhaled β-agonists or nitric oxide, length of hospital stay, days on ventilator, and death.
  
  
  RESULTS
  The CD versus the no-CD CHD cohorts had similar Risk Adjustment in Congenital Heart Surgery-1 risk categories, repair track, age at surgery, and follow-up evaluation times. Respiratory complications (76% vs 37%; P = .006), need for tracheostomy (16% vs 0%; P = .047), and use of inhaled β-agonists (64% vs 11%; P = .0001) all were increased significantly in heterotaxy-CHD patients with CD. No significant differences were detected in postoperative hospital stay, days on mechanical ventilation, or surgical mortality. A trend toward increased mortality for the CD group beyond the postoperative period was observed (33% vs 0%; P = .055) in patients younger than age 10 years.
  
  
  CONCLUSIONS
  Our findings showed that heterotaxy-CHD patients with CD may have increased risks for respiratory deficiencies. Overall, there was a trend toward increased mortality in CD patients with intermediate follow-up evaluation. Because β-agonists are known to increase ciliary beat frequency, presurgical screening for CD and perioperative treatment of CD patients with inhaled β-agonists may improve postoperative outcomes and survival.
authors.affiliation: Children's National Heart Institute, Children's National Medical Center, Washington, DC. Electronic address: hardenb@kidsheart.com.
authors.forename: Brandon
authors.initials: B
authors.lastname: Harden
authors.affiliation: Office of Biostatistics Research, National Institutes of Health, Bethesda, Md.
authors.forename: Xin
authors.initials: X
authors.lastname: Tian
authors.affiliation: Howard Hughes Medical Institute, National Institutes of Health, Bethesda, Md.
authors.forename: Rachel
authors.initials: R
authors.lastname: Giese
authors.affiliation: Department of Pediatric Pulmonology, Children's National Medical Center, Washington, DC.
authors.forename: Nader
authors.initials: N
authors.lastname: Nakhleh
authors.affiliation: Department of Pediatric Pulmonology, Children's National Medical Center, Washington, DC.
authors.forename: Safina
authors.initials: S
authors.lastname: Kureshi
authors.affiliation: Department of Developmental Biology, University of Pittsburgh School of Medicine, Pittsburgh, Pa.
authors.forename: Richard
authors.initials: R
authors.lastname: Francis
authors.affiliation: Children's National Heart Institute, Children's National Medical Center, Washington, DC.
authors.forename: Sridhar
authors.initials: S
authors.lastname: Hanumanthaiah
authors.affiliation: Department of Developmental Biology, University of Pittsburgh School of Medicine, Pittsburgh, Pa.
authors.forename: You
authors.initials: Y
authors.lastname: Li
authors.affiliation: Howard Hughes Medical Institute, National Institutes of Health, Bethesda, Md.
authors.forename: Matthew
authors.initials: M
authors.lastname: Swisher
authors.affiliation: Children's National Heart Institute, Children's National Medical Center, Washington, DC.
authors.forename: Karen
authors.initials: K
authors.lastname: Kuehl
authors.affiliation: Department of Pediatric Pulmonology, Children's National Medical Center, Washington, DC.
authors.forename: Iman
authors.initials: I
authors.lastname: Sami
authors.affiliation: National Institute of Allergy and Infectious Disease, National Institutes of Health, Bethesda, Md.
authors.forename: Kenneth
authors.initials: K
authors.lastname: Olivier
authors.affiliation: Children's National Heart Institute, Children's National Medical Center, Washington, DC.
authors.forename: Richard
authors.initials: R
authors.lastname: Jonas
authors.affiliation: Department of Developmental Biology, University of Pittsburgh School of Medicine, Pittsburgh, Pa.
authors.forename: Cecilia W
authors.initials: CW
authors.lastname: Lo
authors.affiliation: Children's National Heart Institute, Children's National Medical Center, Washington, DC.
authors.forename: Linda
authors.initials: L
authors.lastname: Leatherbury
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002925:Ciliary Motility Disorders / Q000150:complications*; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000601:surgery; D059446:Heterotaxy Syndrome / Q000150:complications* / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000453:epidemiology* / Q000209:etiology*; D011446:Prospective Studies; D012120:Respiration Disorders / Q000453:epidemiology* / Q000209:etiology*; D016896:Treatment Outcome; D055815:Young Adult