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Am J Perinatol 2015; 32(09): 887-894
DOI: 10.1055/s-0035-1544949
Original Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Management Practice and Mortality for Infants with Congenital Diaphragmatic Hernia

Jonathan R. Malowitz
1   Department of Pediatrics, Duke University Medical Center, Durham, North Carolina
2   Duke Clinical Research Institute, Duke University Medical Center, Durham, North Carolina
,
Christoph P. Hornik
1   Department of Pediatrics, Duke University Medical Center, Durham, North Carolina
2   Duke Clinical Research Institute, Duke University Medical Center, Durham, North Carolina
,
Matthew M. Laughon
3   Department of Pediatrics, North Carolina Children's Hospital, Chapel Hill, North Carolina
,
Daniela Testoni
2   Duke Clinical Research Institute, Duke University Medical Center, Durham, North Carolina
4   Division of Neonatal Medicine, Escola Paulista de Medicina – Universidade Federal de São Paulo, São Paulo, Brazil
,
C. Michael Cotten
1   Department of Pediatrics, Duke University Medical Center, Durham, North Carolina
,
Reese H. Clark
5   Pediatrix Medical Group, Sunrise, Florida
,
P. Brian Smith
1   Department of Pediatrics, Duke University Medical Center, Durham, North Carolina
2   Duke Clinical Research Institute, Duke University Medical Center, Durham, North Carolina
› Author Affiliations
Further Information

Publication History

27 June 2014

19 December 2014

Publication Date:
25 February 2015 (online)

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Abstract

Objective Congenital diaphragmatic hernia (CDH) is fatal in 20 to 40% of cases, largely due to pulmonary dysmaturity, lung hypoplasia, and persistent pulmonary hypertension. Evidence for survival benefit of inhaled nitric oxide (iNO), extracorporeal membrane oxygenation (ECMO), and other medical interventions targeting pulmonary hypertension is lacking. We assessed medical interventions and mortality over time in a large multicenter cohort of infants with CDH.

Study Design We identified all infants ≥ 34 weeks' gestation with CDH discharged from 29 neonatal intensive care units between 1999 and 2012 with an average of ≥ 2 CDH admissions per year. We examined mortality and the proportion of infants exposed to medical interventions, comparing four periods of time: 1999–2001, 2002–2004, 2005–2007, and 2008–2012.

Results We identified 760 infants with CDH. From 1999–2001 to 2008–2012, use of iNO increased from 20% of infants to 50%, sildenafil use increased from 0 to 14%, and milrinone use increased from 0 to 22% (p < 0.001). Overall mortality (28%) did not significantly change over time compared with the earliest time period.

Conclusion Despite changing use of iNO, sildenafil, and milrinone, CDH mortality has not significantly decreased in this population of infants.

Keywords

infants - congenital diaphragmatic hernia - lung dysmaturity - lung hypoplasia - pulmonary hypertension - respiratory management
 

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