Pulmonary Hypertension in Patients with Congenital Diaphragmatic Hernia: Does Lung Size Matter?

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,  < 0.01) and o:eLHR (OR = 0.97, 95% CI = 0.94-0.99,  < 0.01) were significantly associated with PHTN grade. Among patients on ECMO, PPLV (OR = 0.92, 95% CI = 0.84-0.99,  = 0.03) and o:eLHR (OR = 0.95, 95% CI = 0.92-0.99,  = 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,  = 0.01 and OR = 0.94, 95% CI = 0.91-0.98,  < 0.01, respectively) and epoprostenol (OR = 0.91, 95% CI = 0.84-0.99,  = 0.02 and OR = 0.93, 95% CI = 0.89-0.98,  < 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.