Title

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

Document Type

Article

Publication Date

12-1-2018

Publication Title

European journal of pediatric surgery

Abstract

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

PubMed ID

29036736

Volume

28

Issue

6

First Page

508

Last Page

514

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