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Pulmonary Hypertension in Patients with Congenital Diaphragmatic Hernia: Does Lung Size Matter?
Journal article   Peer reviewed

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

Arin L Madenci, Joseph T Church, Robert J Gajarski, Kathryn Marchetti, Edwin J Klein, Megan A Coughlin, Jeannie Kreutzmann, Marjorie Treadwell, Maria Ladino-Torres and George B Mychaliska
European journal of pediatric surgery, Vol.28(6), pp.508-514
12/01/2018
DOI: 10.1055/s-0037-1607291
PMCID: PMC7183369
PMID: 29036736
url
https://www.ncbi.nlm.nih.gov/pmc/articles/7183369View
Open Access

Abstract

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.  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.  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).  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.
 Abnormalities, Multiple - diagnostic imaging Echocardiography Female Follow-Up Studies Hernias, Diaphragmatic, Congenital - complications Hernias, Diaphragmatic, Congenital - diagnostic imaging Humans Hypertension, Pulmonary - diagnosis Hypertension, Pulmonary - etiology Infant, Newborn Logistic Models Lung - abnormalities Lung - diagnostic imaging Lung Diseases - complications Lung Diseases - diagnostic imaging Male Pregnancy Retrospective Studies Severity of Illness Index Ultrasonography, Prenatal

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