title: Severe left diaphragmatic hernia limits size of fetal left heart more than does right diaphragmatic hernia.
abstract: OBJECTIVE
  To assess whether severity of congenital diaphragmatic hernia (CDH) correlates with the degree of left heart hypoplasia and left ventricle (LV) output, and to determine if factors leading to abnormal fetal hemodynamics, such as compression and reduced LV preload, contribute to left heart hypoplasia.
  
  
  METHODS
  This was a retrospective cross-sectional study of fetuses at 16-37 weeks' gestation that were diagnosed with CDH between 2000 and 2010. Lung-to-head ratio (LHR), liver position and side of the hernia were determined from stored ultrasound images. CDH severity was dichotomized based on LHR and liver position. The dimensions of mitral (MV) and aortic (AV) valves and LV were measured, and right and left ventricular outputs were recorded.
  
  
  RESULTS
  In total, 188 fetuses with CDH were included in the study, 171 with left CDH and 17 with right CDH. Fetuses with severe left CDH had a smaller MV (Z = -2.24 ± 1.3 vs -1.33 ± 1.08), AV (Z = -1.39 ± 1.21 vs -0.51 ± 1.05) and LV volume (Z = -4.23 ± -2.71 vs -2.08 ± 3.15) and had lower LV output (26 ± 10% vs 32 ± 10%) than those with mild CDH. MV and AV in fetuses with right CDH (MV, Z = -0.83 ± 1.19 and AV, Z = -0.71 ± 1.07) were larger than those in fetuses with left CDH, but LV outputs were similarly diminished, regardless of hernia side. Severe dextroposition and abnormal liver position were associated independently with smaller left heart, while LHR was not.
  
  
  CONCLUSIONS
  The severity of left heart hypoplasia correlates with the severity of CDH. Altered fetal hemodynamics, leading to decreased LV output, occurs in both right- and left-sided CDH, but the additional compressive effect on the left heart is seen only when the hernia is left-sided. Improved knowledge of the physiology of this disease may lead to advances in therapy and better risk assessment for use in counseling affected families.
authors.affiliation: Department of Pediatrics, Divisions of Cardiology, University of California, Benioff Children's Hospital, San Francisco, CA, USA.
authors.forename: F A
authors.initials: FA
authors.lastname: Byrne
authors.affiliation: Department of Pediatrics, Division of Neonatology, University of California, Benioff Children's Hospital, San Francisco, CA, USA.
authors.forename: R L
authors.initials: RL
authors.lastname: Keller
authors.affiliation: Department of Pediatrics, Divisions of Cardiology, University of California, Benioff Children's Hospital, San Francisco, CA, USA.
authors.forename: J
authors.initials: J
authors.lastname: Meadows
authors.affiliation: Department of Surgery, Division of Pediatric Surgery, University of California, San Francisco, CA, USA.|Fetal Treatment Center, University of California, San Francisco, CA, USA.
authors.forename: D
authors.initials: D
authors.lastname: Miniati
authors.affiliation: Department of Pediatrics, Divisions of Cardiology, University of California, Benioff Children's Hospital, San Francisco, CA, USA.
authors.forename: M M
authors.initials: MM
authors.lastname: Brook
authors.affiliation: Department of Pediatrics, Divisions of Cardiology, University of California, Benioff Children's Hospital, San Francisco, CA, USA.
authors.forename: N H
authors.initials: NH
authors.lastname: Silverman
authors.affiliation: Department of Pediatrics, Divisions of Cardiology, University of California, Benioff Children's Hospital, San Francisco, CA, USA.|Fetal Treatment Center, University of California, San Francisco, CA, USA.
authors.forename: A J
authors.initials: AJ
authors.lastname: Moon-Grady
mesh_terms: D003430:Cross-Sectional Studies; D004452:Echocardiography / Q000379:methods; D005260:Female; D047109:Fetal Development; D005315:Fetal Diseases / Q000000981:diagnostic imaging*; D005318:Fetal Heart / Q000033:anatomy & histology / Q000000981:diagnostic imaging*; D005865:Gestational Age; D006257:Head / Q000000981:diagnostic imaging / Q000196:embryology; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000196:embryology; D065630:Hernias, Diaphragmatic, Congenital / Q000150:complications / Q000000981:diagnostic imaging / Q000196:embryology*; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000000981:diagnostic imaging / Q000196:embryology*; D008099:Liver / Q000000981:diagnostic imaging / Q000196:embryology; D008168:Lung / Q000000981:diagnostic imaging / Q000196:embryology; D009929:Organ Size; D011247:Pregnancy; D012189:Retrospective Studies; D016216:Ultrasonography, Prenatal / Q000379:methods
keywords: cardiac development; congenital diaphragmatic hernia; congenital heart defects; fetal cardiac output; left heart hypoplasia