title: Perinatal course of Ebstein's anomaly and tricuspid valve dysplasia in the fetus.
abstract: OBJECTIVE
  We sought to better define echocardiographic predictors of perinatal mortality in fetuses with Ebstein's anomaly (EA) or tricuspid valve dysplasia (TVD).
  
  
  METHODS
  Parameters included measured chamber size, the presence of hydrops, and Doppler recordings of the left ventricular (LV) myocardial performance index (MPI).
  
  
  RESULTS
  Between 1 January 2000 and 31 December 2008, 21 fetuses were diagnosed with either EA (17) or TVD (4). Five fetuses were lost to follow-up, and 12 of 16 fetuses were born live (75%). Survivors were found to have smaller right atrial area index scores when compared with non-survivors (1.025 ± 0312 vs 1.502 ± 0.105, respectively, p = 0.013) and were less likely to present with hydrops (0% survivors vs 75% of non-survivors, p < 0.01). LV MPI sub-analysis revealed a shorter combined isovolemic contraction and relaxation time for non-survivors compared wth survivors (46.5 ± 8.2 ms vs 82.3 ± 21.2 ms, respectively, p = 0.004) although no difference was observed for LV ejection times or overall LV MPI between survivors and non-survivors.
  
  
  CONCLUSIONS
  Physiologic analysis of left ventricular function via the LV Tei index and its component measurements demonstrates potentially novel insights into hemodynamic derangements and their association with outcomes in patients with EA/TVD.
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA. lasaj@email.chop.edu
authors.forename: Javier J
authors.initials: JJ
authors.lastname: Lasa
authors.forename: Zhi-Yun
authors.initials: ZY
authors.lastname: Tian
authors.forename: Rong
authors.initials: R
authors.lastname: Guo
authors.forename: Jack
authors.initials: J
authors.lastname: Rychik
mesh_terms: D000328:Adult; D004437:Ebstein Anomaly / Q000175:diagnosis* / Q000453:epidemiology / Q000401:mortality; D005260:Female; D005315:Fetal Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000401:mortality; D005333:Fetus / Q000473:pathology; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000453:epidemiology / Q000401:mortality; D006349:Heart Valve Diseases / Q000151:congenital / Q000175:diagnosis* / Q000453:epidemiology / Q000401:mortality; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D050498:Live Birth / Q000453:epidemiology; D054238:Perinatal Mortality; D011247:Pregnancy; D011379:Prognosis; D017741:Survivors / Q000706:statistics & numerical data; D014261:Tricuspid Valve / Q000002:abnormalities*; D016216:Ultrasonography, Prenatal; D055815:Young Adult