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Eur J Pediatr Surg 2018; 28(06): 508-514
DOI: 10.1055/s-0037-1607291
Original Article
Georg Thieme Verlag KG Stuttgart · New York

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

Arin L. Madenci   *
1   Department of Surgery, Brigham and Women's Hospital, Boston, Massachusetts, United States
,
Joseph T. Church   *
2   Department of Surgery, University of Michigan Health System, Ann Arbor, Michigan, United States
,
Robert J. Gajarski
3   Department of Pediatric Cardiology, Nationwide Children's Hospital, Columbus, Ohio, United States
,
Kathryn Marchetti
4   Department of Education, University of Michigan Medical School, Ann Arbor, Michigan, United States
,
Edwin J. Klein
4   Department of Education, University of Michigan Medical School, Ann Arbor, Michigan, United States
,
Megan A. Coughlin
5   Department of Surgery, Henry Ford Health System, Detroit, Michigan, United States
,
Jeannie Kreutzmann
6   Section of Pediatric Surgery, Department of Surgery, University of Michigan Medical School and C.S. Mott Children's Hospital, Ann Arbor, Michigan, United States
,
Marjorie Treadwell
7   Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of Michigan Medical School and C.S. Mott Children's Hospital, Ann Arbor, Michigan, United States
,
Maria Ladino-Torres
8   Section of Pediatric Radiology, Department of Radiology, University of Michigan Medical School and C.S. Mott Children's Hospital, Ann Arbor, Michigan, United States
,
George B. Mychaliska
6   Section of Pediatric Surgery, Department of Surgery, University of Michigan Medical School and C.S. Mott Children's Hospital, Ann Arbor, Michigan, United States
› Author Affiliations
Further Information

Publication History

15 June 2017

05 September 2017

Publication Date:
16 October 2017 (online)

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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, 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.

Keywords

congenital diaphragmatic hernia - pulmonary hypertension - pulmonary hypoplasia - prenatal ultrasonography - magnetic resonance imaging

* Both the authors contributed equally to the article.