Objective
Congenital diaphragmatic hernia (CDH) results in morbidity and mortality due to 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.
Study design
We conducted a retrospective study of fetuses with an antenatal ultrasound and left-sided CDH cared for at the University of California San Francisco (2002–12). Fetal liver position was classified on ultrasound 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 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 using 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 (LHR model only), with censoring at 100 days.
Results
Of 118 patients, fetal markers were available as follows: LHR (n=53), liver position (n=112), and stomach position (n=80). Fewer infants resolved PH if they had LHR<1 (p=0.006), thoracic liver position (p=0.001), or more aberrant stomach position (p<0.001). There was also a decreased rate of resolution of PH in infants with LHR <1 (hazard ratio 0.30, p=0.007), thoracic liver position (hazard ratio 0.38, p<0.001), and more aberrant stomach position (hazard ratios 0.28, p=0.002; 0.1, p<0.001; 0.07 p<0.001).
Conclusion
Fetal ultrasound markers of CDH severity are predictive not just of mortality 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.