Pulmonary Hypertension in Patients with Congenital Diaphragmatic Hernia: Does Lung Size Matter?
Madenci AL, Church JT, Gajarski RJ, Marchetti K, Klein EJ, Coughlin MA, Kreutzmann J, Treadwell M, Ladino-Torres M, and Mychaliska GB. Pulmonary hypertension in patients with congenital diaphragmatic hernia: Does lung size matter? Eur J Pediatr Surg 2018; 28(6):508-514.
European journal of pediatric surgery
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,
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.
Medical Subject Headings
Abnormalities, Multiple; Echocardiography; Female; Follow-Up Studies; Hernias, Diaphragmatic, Congenital; Humans; Hypertension, Pulmonary; Infant, Newborn; Logistic Models; Lung; Lung Diseases; Male; Pregnancy; Retrospective Studies; Severity of Illness Index; Ultrasonography, Prenatal