title: Congenital heart anomaly in newborns with congenital diaphragmatic hernia: a single-center experience.
abstract: OBJECTIVE
  To evaluate the impact of the presence of a congenital heart anomaly (CHA) and its potential contribution to morbidity and mortality in infants with congenital diaphragmatic hernia (CDH).
  
  
  METHODS
  In this retrospective cohort study, prenatal and postnatal data of all newborns diagnosed with CDH between January 2004 and December 2012 in a single center were reviewed. Cases were classified into two groups: those with 'isolated' CDH and those with both CDH and CHA. Patients with CHA were further subclassified into those with a major or minor CHA based on the Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), and the Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STS-EACTS) scoring systems. Patients with associated non-cardiac anomalies, including 'syndromic cases', were excluded from the analysis. Primary and secondary outcomes were survival up to 1 year of age and a need for extracorporeal membrane oxygenation (ECMO), respectively.
  
  
  RESULTS
  Of the 180 infants with CDH, 41 were excluded because of the presence of non-cardiac associated anomalies, 118 had isolated CDH and 21 had CDH with CHA (16 with minor and five with major CHA). Receiver-operating characteristics curve analysis demonstrated that the best cut-off for survival was when the score for CHA was ≤ 2 for both RACHS-1 (area under the curve (AUC), 0.74 (P = 0.04); sensitivity, 80.0%; specificity, 87.5%) and STS-EACTS (AUC, 0.83 (P = 0.03); sensitivity, 100%; specificity, 87.5%). Survival rate at 1 year was significantly lower in those with CHD and a major CHA (40.0%; P = 0.04) than in those with isolated CDH (77.1%) and those with CDH and a minor CHA (81.3%). We found no significant differences among the groups with regard to the need for ECMO.
  
  
  CONCLUSIONS
  In general, a milder form of CHA does not appear to have a negative impact on the survival of infants with CDH. However, mortality appears to be significantly higher in infants with CDH and a major form of CHA. The scoring systems appear to be useful as predictors for classifying the effects of CHA in this population of patients.
authors.affiliation: Department of Obstetrics and Gynecology, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: R
authors.initials: R
authors.lastname: Ruano
authors.affiliation: Department of Obstetrics and Gynecology, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: P
authors.initials: P
authors.lastname: Javadian
authors.affiliation: Department of Pediatrics, Division of Cardiology, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: J A
authors.initials: JA
authors.lastname: Kailin
authors.affiliation: Department of Pediatrics, Division of Cardiology, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: S A
authors.initials: SA
authors.lastname: Maskatia
authors.affiliation: Department of Obstetrics and Gynecology, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: A A
authors.initials: AA
authors.lastname: Shamshirsaz
authors.affiliation: Michael E. DeBakey Department of Surgery, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: D L
authors.initials: DL
authors.lastname: Cass
authors.affiliation: Michael E. DeBakey Department of Surgery, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: I J
authors.initials: IJ
authors.lastname: Zamora
authors.affiliation: Department of Obstetrics and Gynecology, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: H
authors.initials: H
authors.lastname: Sangi-Haghpeykar
authors.affiliation: Michael E. DeBakey Department of Surgery, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: T C
authors.initials: TC
authors.lastname: Lee
authors.affiliation: Department of Pediatrics, Division of Cardiology, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: N A
authors.initials: NA
authors.lastname: Ayres
authors.affiliation: Department of Radiology, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: A
authors.initials: A
authors.lastname: Mehollin-Ray
authors.affiliation: Department of Radiology, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: C I
authors.initials: CI
authors.lastname: Cassady
authors.affiliation: Department of Pediatrics, Division of Neonatology, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: C
authors.initials: C
authors.lastname: Fernandes
authors.affiliation: Department of Pediatrics, Division of Neonatology, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: S
authors.initials: S
authors.lastname: Welty
authors.affiliation: Department of Obstetrics and Gynecology, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: M A
authors.initials: MA
authors.lastname: Belfort
authors.affiliation: Michael E. DeBakey Department of Surgery, Texas Children's Fetal Center and Baylor College of Medicine, Houston, TX, USA.
authors.forename: O O
authors.initials: OO
authors.lastname: Olutoye
mesh_terms: D015199:Extracorporeal Membrane Oxygenation / Q000706:statistics & numerical data; D006330:Heart Defects, Congenital / Q000145:classification / Q000150:complications / Q000401:mortality*; D065630:Hernias, Diaphragmatic, Congenital / Q000150:complications / Q000401:mortality*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D012372:ROC Curve; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D015996:Survival Rate
keywords: ECMO; cardiac anomaly; chromosomal anomaly; congenital diaphragmatic hernia; lung-to-head ratio; prenatal diagnosis; pulmonary hypoplasia; survival