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; George B. Mychaliska

doi:10.1055/s-0037-1607291

European Journal of Pediatric Surgery, Inhaltsverzeichnis

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?

Autoren

Arin L. Madenci^*

¹Department of Surgery, Brigham and Women's Hospital, Boston, Massachusetts, United States
Joseph T. Church^*

²Department of Surgery, University of Michigan Health System, Ann Arbor, Michigan, United States
Robert J. Gajarski

³Department of Pediatric Cardiology, Nationwide Children's Hospital, Columbus, Ohio, United States
Kathryn Marchetti

⁴Department of Education, University of Michigan Medical School, Ann Arbor, Michigan, United States
Edwin J. Klein

⁴Department of Education, University of Michigan Medical School, Ann Arbor, Michigan, United States
Megan A. Coughlin

⁵Department of Surgery, Henry Ford Health System, Detroit, Michigan, United States
Jeannie Kreutzmann

⁶Section 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

⁷Division 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

⁸Section 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

⁶Section of Pediatric Surgery, Department of Surgery, University of Michigan Medical School and C.S. Mott Children's Hospital, Ann Arbor, Michigan, United States

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

Volltext

Referenzen

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