1. Variability in the Delivery Room Management of Infants with Congenital Anomalies Heather M. French, MD Perelman School of Medicine at the University of Pennsylvania The Children’s Hospital of Philadelphia

2. Faculty Disclosure Information In the past 12 months, I have no relevant financial relationships with the manufacturer(s) of any commercial product(s) and/or provider(s) of commercial services discussed in this CME activity. I do not intend to discuss an unapproved/investigative use of a commercial product/device in my presentation.

3. Objectives: 1.Describe the variability in DR management of infants with congenital anomalies that exists across the United States 2.Discuss common morbidities noted in the acute post-delivery stabilization period of infants with congenital anomalies 3.Introduce the need for development of standardized delivery room protocols to minimize early morbidity and mortality for infants with prenatally-diagnosed common congenital anomalies

4. Good News! The infant mortality rate caused by congenital anomalies is improving! Nervous system malformations Cardiovascular malformations Gastrointestinal malformations Chromosomal malformations Lee et al, Obstet Gynecol 2001

5. CDC: Neonatal Mortality Rate, 1999-2010 Cause of death (ICD-9)Rate per 100,000 deaths Prematurity and low birth weight106.0 Birth defects (Q00-Q99)92.1 Heart defect14.3 Anencephaly7.6 CDC Health Data Interactive, accessed 09.18.14

6. Hypothesis Significant variability in DR management of infants with congenital anomalies exists across the United States. This variability can be identified and described by performing a literature review and national survey.

7. Specific Aims 1.Perform a systematic literature review of DR resuscitation guidelines for infants with prenatally diagnosed congenital anomalies. 2.Conduct a survey of NICU medical directors across the US to characterize the variability in DR management of infants with common congenital anomalies.

8. Systematic Literature Review Focused on CDH, CHD, gastroschisis, omphalocele, myelomeningocele MeSH search terms – resuscitation, delivery room, algorithm, clinical protocol, disease management

9. Literature Review Results Best described for CHD and CDH 2 papers for CHD CDH – “gentle ventilation”; most focus on NICU management pre-repair Abdominal wall defects – “prevent heat loss, cover defect”

10. “Data is available on the ‘best practices’ for initial resuscitation and newborn case for many common anomalies”

11. Electronic survey via Survey Monkey

12. Electronic survey Response rate – 31.4%

13. Question for the audience: Do you perceive there to be variability of DR practices for infants with congenital anomalies within your own institution?

15. Congenital Diaphragmatic Hernia Even with protocols in place Parameters for Max PIP/PEEP, MAP, initial FiO 2, pulse-ox goals are highly variable across centers Variable use of iNO, HFOV, surfactant, PGE 1, paralysis, sedation in the DR

16. Congenital Diaphragmatic Hernia

20. Congenital Heart Disease 32% of respondents come from an institution with DR protocols 83% of institutions that have CHD DR protocols stratify by type of cardiac lesion for management

21. Congenital Heart Disease

24. Variable practices noted for: Gastroschisis & omphalocele positioning Management of bowel in gastroschisis Management of omphalocele sac Management of MMC defect

25. Gastroschisis

27. ”When standards of care in medicine are clear, practice patterns are similar in every part of the country. When there is no clear evidence on the best practices, however, different physicians will adopt different approaches, on the basis of their beliefs, training, incentives, and the local ‘practice style’.” JACC 2013; 62, 5

28. Conclusions Significant variability exists in DR management for infants with congenital anomalies Significant DR morbidities are reported Without standards of care, we cannot examine morbidities and study our practices in any systematic way

29. Future Directions Creation of DR guidelines based on available evidence and Delphi process Vigorous data collection to better understand DR morbidity and mortality frequency

30. Acknowledgements CHOP Anne Ades, MD Annie Giaccone, MD Natalie Rintoul, MD St. Christopher’s Hospital Endla Anday, MD Westat Biostatistics Okan Elci, PhD

32. Extra slides

33. NRP Current Issues Seminar: NRP Grant Research Presentation 1 Heather French, MD, FAAP Children’s Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania; Philadelphia, PA

34. Classification of neonatal deaths, 1998- 2009 Admitted Not admitted

35. CDC Infant Mortality Rates

37. Morbidity with CDH Deliveries