title: Vasoreactive response to maternal hyperoxygenation in the fetus with hypoplastic left heart syndrome.
abstract: BACKGROUND
  Cardiopulmonary interactions play an important role in the pathophysiology of hypoplastic left heart syndrome (HLHS). Pulmonary vasculopathy has been identified, especially in those with restrictive/intact atrial septum. Responsiveness of the pulmonary vasculature to maternal hyperoxygenation (MH) may provide a tool to assess the degree of pulmonary vasculopathy present before birth.
  
  
  RESULTS
  Doppler echocardiography was performed in 27 normal and 43 HLHS fetuses. In HLHS, sampling was repeated after 10 minutes of MH with 60% FiO(2) and after 5 minutes of recovery. Sampling was performed in the proximal, midportion, and distal branch pulmonary artery (PA). Pulsatility index (PI) was used as a measure of vascular impedance. Of the HLHS fetuses, 34 had an open interatrial septum and 9 had a restrictive/intact atrial septum. At birth, 5 fetuses underwent immediate intervention on the interatrial septum. Middle cerebral artery PI was lower in HLHS versus normal fetuses (P<0.001). There was no difference in UA, DA, or branch PA PI between normal fetuses and those with HLHS. MH led to a significant decrease in PI at each of the PA sites sampled in fetuses with an open atrial septum (P<0.001); however, there no was significant change in the PI in fetuses that required immediate intervention on the atrial septum at birth. Using a cutoff value of <10% vasoreactivity, the sensitivity of MH testing for determining need for immediate intervention at birth is 100% (0.46 to 1.0); specificity, 94% (0.78 to 0.99); positive predictive value, 71% (0.30 to 0.95); and negative predictive value, 100% (0.86 to 1.0). No untoward effects were seen with MH.
  
  
  CONCLUSIONS
  PA vasoreactivity to MH occurs in the fetus with HLHS. MH testing accurately identifies fetuses requiring urgent postnatal intervention at birth and may be used to select candidates for fetal atrial septoplasty.
authors.affiliation: The Fetal Heart Program at Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. szwast@email.chop.edu
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: Denise
authors.initials: D
authors.lastname: Donaghue
authors.forename: Jack
authors.initials: J
authors.lastname: Rychik
mesh_terms: D000704:Analysis of Variance; D003430:Cross-Sectional Studies; D015150:Echocardiography, Doppler; D005260:Female; D005318:Fetal Heart / Q000000981:diagnostic imaging / Q000503:physiopathology; D005323:Fetal Monitoring; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D010100:Oxygen / Q000097:blood*; D011237:Predictive Value of Tests; D011247:Pregnancy; D011446:Prospective Studies; D011651:Pulmonary Artery / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D011673:Pulsatile Flow; D012680:Sensitivity and Specificity; D016216:Ultrasonography, Prenatal / Q000379:methods*
chemical_list: D010100:Oxygen