European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie · 2017
Pulmonary Hypertension in Patients with Congenital Diaphragmatic Hernia: Does Lung Size Matter?
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Madenci Arin L, Church Joseph T, Gajarski Robert J, Marchetti Kathryn, Klein Edwin J, Coughlin Megan A, Kreutzmann Jeannie, Treadwell Marjorie, Ladino-Torres Maria, Mychaliska George B
Аннотация
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.
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.