title: Right ventricular mechanics in the fetus with hypoplastic left heart syndrome.
abstract: BACKGROUND
  Right ventricular mechanics influence outcomes in patients with hypoplastic left heart syndrome (HLHS). The aim of this study was to determine whether differences in right ventricular performance have their origins in fetal life and if the architectural character of the hypoplastic left ventricle affects right ventricular mechanics.
  
  
  METHODS
  The first complete fetal echocardiograms after 17 weeks' gestation were reviewed in 84 fetuses with HLHS and in 115 gestational age-matched normal controls. Inflow, outflow, and myocardial tissue Doppler velocities were measured. E/A and E/e' ratios and right ventricular myocardial performance index were calculated.
  
  
  RESULTS
  In fetuses with HLHS, there were lower tricuspid E/A ratios (mean, 0.6 ± 0.1 vs 0.7 ± 0.1; P < .001), higher E/e' ratios (mean, 8.1 ± 2.6 vs 7.0 ± 1.3; P = .006), and higher right ventricular myocardial performance indices (mean, 0.47 ± 0.14 vs 0.40 ± 0.10; P < .001) compared with controls. Among fetuses with HLHS grouped according to left ventricular architecture, those with left ventricular endocardial fibroelastosis had the most striking differences in right ventricular mechanics.
  
  
  CONCLUSIONS
  Right ventricular mechanics are different from normal in fetuses with HLHS and are influenced by the presence of left ventricular endocardial fibroelastosis. These differences precede the imposition of undue loading conditions as a consequence of surgical palliation and may offer clues to the development of later right ventricular failure.
authors.affiliation: Fetal Heart Program, Cardiac Center at The Children's Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA, USA. natarajans@email.chop.edu
authors.forename: Shobha
authors.initials: S
authors.lastname: Natarajan
authors.forename: Anita
authors.initials: A
authors.lastname: Szwast
authors.forename: Zhiyun
authors.initials: Z
authors.lastname: Tian
authors.forename: Margaret
authors.initials: M
authors.lastname: McCann
authors.forename: Debbra
authors.initials: D
authors.lastname: Soffer
authors.forename: Jack
authors.initials: J
authors.lastname: Rychik
mesh_terms: D003430:Cross-Sectional Studies; D015150:Echocardiography, Doppler; D005260:Female; D005333:Fetus; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000196:embryology* / Q000503:physiopathology; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000000981:diagnostic imaging / Q000196:embryology* / Q000503:physiopathology; D011247:Pregnancy; D012189:Retrospective Studies; D016216:Ultrasonography, Prenatal