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
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.
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
Nervous system
malformations
Cardiovascular
malformations
Gastrointestinal
malformations
Chromosomal
malformations
Good News!
The infant mortality
rate caused by
congenital anomalies
is improving!
Lee et al, Obstet Gynecol 2001
CDC: Neonatal Mortality Rate,
1999-2010
Cause of death (ICD-9)
Rate per 100,000 deaths
Prematurity and low birth weight
106.0
Birth defects (Q00-Q99)
92.1
Heart defect
14.3
Anencephaly
7.6
CDC Health Data Interactive, accessed 09.18.14
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.
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.
Systematic Literature Review
• Focused on CDH, CHD, gastroschisis,
omphalocele, myelomeningocele
• MeSH search terms – resuscitation,
delivery room, algorithm, clinical protocol,
disease management
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”
“Data is available on the
‘best practices’ for initial
resuscitation and
newborn case for many
common anomalies”
Electronic survey via Survey Monkey
Electronic survey
Response rate – 31.4%
Respondents by Practice
Type (%)
Respondents by US region (%)
Private practice, not
academic
9.3
22.5
Private practice,
academic
West
20
31
Midwest
Academic, not private
practice
11
Northeast
Hospital employed
23
56.7
South
26
Question for the audience:
Do you perceive there to be variability of DR
practices for infants with congenital
anomalies within your own institution?
Do you perceive
variability in DR
management strategies
within your institution?
Does your institution have a
DR protocol for…
80
70
80
60
70
50
60
40
50
30
Yes
40
20
No
30
10
20
0
10
0
Yes
No
Congenital Diaphragmatic Hernia
Even with protocols in place
• Parameters for Max PIP/PEEP, MAP, initial
FiO2, pulse-ox goals are highly variable
across centers
• Variable use of iNO, HFOV, surfactant, PGE1,
paralysis, sedation in the DR
Congenital Diaphragmatic Hernia
Max PIP in the DR
70
60
% of respondents
Yes protocol
50
No protocol
40
30
20
10
0
<20
20-22
23-25
mmHg
26-28
>28
Congenital Diaphragmatic Hernia
Initial FiO2 in DR
45
% of respondents
40
35
Yes protocol
30
No protocol
25
20
15
10
5
0
21
22-30
31-50 51-75 76-100
% FiO2
Congenital Diaphragmatic Hernia
Pre-ductal SpO2 goals in the
DR
70
% of respondents
60
50
40
Yes protocol
30
No protocol
20
10
0
> 70
> 80
> 85
SpO2
> 90
> 95
Congenital Diaphragmatic Hernia
pH goals in the DR
50
45
% of respondents
40
35
30
25
Yes protocol
20
No protocol
15
10
5
0
≥ 7.15
≥ 7.2
≥ 7.25 ≥ 7.3
pH
≥ 7.35
≥ 7.4
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
Congenital Heart Disease
Starting Dose of PGE1
50
45
% of respondents
40
35
30
25
20
15
10
5
0
0.01
0.011-0.049
0.05
0.051-0.1
mcg/kg/min
> 0.1
Congenital Heart Disease
Starting FiO2 in the DR
80
% of respondents
70
60
50
40
30
20
10
0
21
22-30
31-50
FiO2
51-75
76-100
Congenital Heart Disease
Goal SpO2 at 10 min of life
80
70
% of respondents
60
50
40
30
20
10
0
70-74
75-85
86-95
SpO2
>95
Variable practices noted for:
• Gastroschisis & omphalocele positioning
• Management of bowel in gastroschisis
• Management of omphalocele sac
• Management of MMC defect
Gastroschisis
Strategies Employed in the DR
to Protect Intestines
70
% of respondents
60
50
40
30
20
10
0
Sterile gloves
Sterile plastic bag
Sterile saline
Sterile gauze
Plastic wrap
Silo placement
inDR
Immediate
reduction
Frequent Morbidities in the Delivery Room
70
60
% of respondents
50
CDH
CHD
40
Gastroschisis
30
Omphalocele
MMC
20
10
0
JACC 2013; 62, 5
”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’.”
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
Future Directions
• Creation of DR guidelines based on
available evidence and Delphi process
• Vigorous data collection to better
understand DR morbidity and mortality
frequency
Acknowledgements
CHOP
Anne Ades, MD
Annie Giaccone, MD
Natalie Rintoul, MD
St. Christopher’s Hospital
Endla Anday, MD
Westat Biostatistics
Okan Elci, PhD
• Extra slides
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
Classification
of neonatal
deaths, 19982009
Admitted
Not admitted
CDC Infant Mortality Rates
Respondents by AAP district (%)
Pacific (CA, OR, WA)
16.8
17.9
Mountain (AZ, CO, ID, MT, NM, NV, UT, WY)
1.7
5.8
West North Central (IA, KS, MN, MO, NE, ND, SD)
East North Central (IL, IN, MI, OH, WI)
6.9
New England (CT, MA, ME, NH, RI, VT)
8.7
Middle Atlantic (NJ, NY, PA)
16.2
19.7
West South Central (AR, LA, OK, TX)
East South Central (AL, KY, MS, TN)
6.4
South Atlantic (DC, DE, FL, GA, MD, NC, SC, VA, WV)
Morbidity with CDH Deliveries
Hypoxemia: Initial FiO2 in the
DR
21
18%
25%
22-30
31-50
8%
7%
42%
51-75
75-100