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Management Practice and Mortality for Infants with Congenital Diaphragmatic Hernia
Jonathan R Malowitz1, Christoph P Hornik1, Matthew M Laughon2
1Department of Pediatrics, Duke University Medical Center, Durham, North Carolina.
Insights
Despite increased use of inhaled nitric oxide (iNO) and other pulmonary hypertension treatments, congenital diaphragmatic hernia (CDH) mortality rates remain unchanged. This study highlights the need for new therapeutic strategies for CDH.
Area of Science:
- Neonatal Medicine
- Pediatric Surgery
- Pulmonary Hypertension Research
Background:
- Congenital diaphragmatic hernia (CDH) presents a significant mortality risk (20-40%) primarily due to pulmonary complications like lung hypoplasia and persistent pulmonary hypertension.
- Existing evidence supporting the survival benefits of inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO) for CDH is limited.
- Medical interventions targeting pulmonary hypertension in CDH infants have evolved, but their impact on survival remains unclear.
Purpose of the Study:
- To evaluate trends in medical interventions for pulmonary hypertension in infants with CDH.
- To assess the association between these interventions and mortality rates in a large, multicenter CDH cohort.
- To analyze changes in treatment strategies and outcomes over distinct time periods.
Main Methods:
- A multicenter cohort study identified 760 infants (≥34 weeks gestation) with CDH admitted between 1999 and 2012.
- Infants were grouped into four time periods for comparative analysis: 1999-2001, 2002-2004, 2005-2007, and 2008-2012.
- Data collected included mortality rates and the proportion of infants receiving specific medical interventions, such as iNO, sildenafil, and milrinone.
Main Results:
- The utilization of inhaled nitric oxide (iNO) significantly increased from 20% in 1999-2001 to 50% in 2008-2012.
- Use of sildenafil and milrinone also rose substantially, from 0% to 14% and 0% to 22%, respectively, during the study period (p < 0.001).
- Despite these shifts in treatment, the overall mortality rate for CDH remained stable at 28% across all analyzed time periods.
Conclusions:
- Increasing use of inhaled nitric oxide (iNO), sildenafil, and milrinone has not led to a significant reduction in CDH-related mortality.
- The findings suggest that current medical interventions targeting pulmonary hypertension may not be sufficient to improve survival in infants with CDH.
- Further research into novel therapeutic approaches is warranted to address the persistent high mortality associated with congenital diaphragmatic hernia.
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.
