title: Fetal ultrasound markers of severity predict resolution of pulmonary hypertension in congenital diaphragmatic hernia.
abstract: OBJECTIVE
  Congenital diaphragmatic hernia (CDH) results in morbidity and death from lung hypoplasia and persistent pulmonary hypertension (PH). We sought to define the relationship between fetal ultrasound markers of severity in CDH and the time to resolution of neonatal PH.
  
  
  METHODS
  We conducted a retrospective study of fetuses with an antenatal ultrasound scan and left-sided CDH cared for at the University of California San Francisco (2002-2012). Fetal liver position was classified on ultrasound scan as abdominal (entire liver within the abdomen) or thoracic (any portion of the liver within the thorax). Fetal stomach position was classified from least to most aberrant: abdominal, anterior left chest, mid-posterior left chest, or retrocardiac (right chest). Lung-to-head ratio (LHR) was determined from available scans at 20-29 weeks of gestational age (GA). Routine neonatal echocardiograms were performed weekly for up to 6 weeks or until PH resolved or until discharge. PH was assessed by echocardiogram with the use of a hierarchy of ductus arteriosus level shunt, interventricular septal position, and tricuspid regurgitant jet velocity. Days to PH-free survival was defined as the age at which pulmonary artery pressure was estimated to be <2/3 systemic blood pressure. Cox proportional hazards models adjusted for GA at birth, era of birth, fetal surgery, and GA at ultrasound scan (LHR model only), with censoring at 100 days.
  
  
  RESULTS
  Of 118 patients, the following fetal markers were available: LHR (n = 53), liver position (n = 112), and stomach position (n = 80). Fewer infants experienced resolved PH if they had LHR <1 (P = .006), thoracic liver position (P = .001), or more aberrant stomach position (P < .001). There was also a decreased rate of resolution of PH in infants with LHR <1 (hazard ratio, 0.30; P = .007), thoracic liver position (hazard ratio, 0.38; P < .001), and more aberrant stomach position (hazard ratios, 0.28 [P = .002]; 0.1 [P < .001]; and 0.07 [P < .001]).
  
  
  CONCLUSIONS
  Fetal ultrasound markers of CDH severity are predictive not only of death but also of significant morbidity. LHR <1, thoracic liver, and aberrant stomach position are associated with delayed time to resolution of PH in infants with CDH and may be used to identify fetuses at high risk of persistent PH.
authors: Division of Neonatology, Department of Pediatrics, Benioff Children's Hospital, University of California, San Francisco, CA. Electronic address: Leslie.Lusk@ucsf.edu.
authors: Leslie A
authors: LA
authors: Lusk
authors: School of Medicine, University of California, San Francisco, CA.
authors: Katherine C
authors: KC
authors: Wai
authors: Fetal Treatment Center, University of California, San Francisco, CA.
authors: Anita J
authors: AJ
authors: Moon-Grady
authors: Department of Radiology and Biomedical Imaging, Medical Center, University of California, San Francisco, CA.
authors: Amaya M
authors: AM
authors: Basta
authors: Department of Radiology and Biomedical Imaging, Medical Center, University of California, San Francisco, CA.
authors: Roy
authors: R
authors: Filly
authors: Division of Neonatology, Department of Pediatrics, Benioff Children's Hospital, University of California, San Francisco, CA; Fetal Treatment Center, University of California, San Francisco, CA.
authors: Roberta L
authors: RL
authors: Keller
mesh_terms: D000328:Adult; D015331:Cohort Studies; D018450:Disease Progression; D004452:Echocardiography; D005260:Female; D005865:Gestational Age; D006257:Head / Q000000981:diagnostic imaging*; D065630:Hernias, Diaphragmatic, Congenital / Q000000981:diagnostic imaging* / Q000401:mortality; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000503:physiopathology; D007231:Infant, Newborn; D008099:Liver / Q000000981:diagnostic imaging*; D008168:Lung / Q000000981:diagnostic imaging*; D008297:Male; D011247:Pregnancy; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013270:Stomach / Q000000981:diagnostic imaging*; D016216:Ultrasonography, Prenatal; D014655:Vascular Resistance
keywords: lung-to-head ratio; pulmonary vascular resistance; ultrasound marker