Pulmonary Hypertension in Patients with Congenital Diaphragmatic Hernia: Does Lung Size Matter?
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In brief
In brief
Retrospective study of 62 infants with isolated CDH examining correlation between prenatal lung size measurements (o:eLHR and PPLV) and postnatal pulmonary hypertension severity. Both metrics significantly predicted PHTN grade and need for therapies like iNO and epoprostenol, with stronger associations in ECMO patients, suggesting prenatal imaging can guide postnatal management.
Written by the GCMD Library team from the article.
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.