Purpose: The relationship between pulmonary hypoplasia and pulmonary arterial hypertension (PHTN) in patients with congenital diaphragmatic hernia (CDH) remains ill-defined. We hypothesized that prenatal estimates of lung size would directly correlate with PHTN severity.
Methods: Infants with isolated CDH (born 2004-2015) at a single institution were included. Estimates of lung size included observed-to-expected LHR (o:eLHR) and %-predicted lung volumes (PPLV = observed/predicted volumes). The primary outcome was severity of PHTN (grade 0-3) on echocardiography performed between day of life 3 and 30.
Results: Among 62 patients included, there was 32% mortality and 65% ECMO utilization. PPLV (odds ratio [OR] = 0.94 per 1 grade in PHTN severity, 95% confidence interval [CI] = 0.89-0.98, p < 0.01) and o:eLHR (OR = 0.97, 95% CI = 0.94-0.99, p < 0.01) were significantly associated with PHTN grade. Among patients on ECMO, PPLV (OR = 0.92, 95% CI = 0.84-0.99, p = 0.03) and o:eLHR (OR = 0.95, 95% CI = 0.92-0.99, p = 0.01) were more strongly associated with PHTN grade. PPLV and o:eLHR were significantly associated with the use of inhaled nitric oxide (iNO) (OR = 0.90, 95% CI = 0.83-0.98, p = 0.01 and OR = 0.94, 95% CI = 0.91-0.98, p < 0.01, respectively) and epoprostenol (OR = 0.91, 95% CI = 0.84-0.99, p = 0.02 and OR = 0.93, 95% CI = 0.89-0.98, p < 0.01, respectively).
Conclusion: Among infants with isolated CDH, PPLV, and o:eLHR were significantly associated with PHTN severity, especially among patients requiring ECMO. Prenatal lung size may help predict postnatal PHTN and associated therapies.
Georg Thieme Verlag KG Stuttgart ยท New York.