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The CDH Registry and Study GroupPast, Present and Future
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CDH Registry
Background of the Registry Current Status Major Publications Future Plans
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5 Neonatologists
7 Pediatric Surgeons
3 Intensivists
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CDH Registry
Existing data forms collated and modified Voluntary collection A priori plan to limit total amount of data Data collection begun 1995 Data in secure, anonymized database Some PHI – DOB, DOS
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The Congenital Diaphragmatic Study Group
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Versions of the CDH Registry• Version 1 1995-2000
oDefining the problem - medications, ventilator strategies, ECLS use
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Version 1
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Version 1
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Versions of the CDH Registry• Version 1 1995-2000
oDefining the problem - medications, ventilator strategies, ECLS use
• Version 2 2001-2006oUnderstanding the details - delivery, oxygen/carbon dioxide, discharge
status, cardiac anomalies
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CDH Registry – Why it has worked Management (2000 – 2019)
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Version 2
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Versions of the CDH Registry• Version 1 1995-2000
oDefining the problem - medications, ventilator strategies, ECLS use
• Version 2 2001-2006oUnderstanding the details - delivery, oxygen/carbon dioxide, discharge
status, cardiac anomalies
• Version 3 2007-2014oStaging - classifying defect size, pulmonary hypertension
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Version 3
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Version 3
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Versions of the CDH Registry• Version 1 1995-2000
oDefining the problem - medications, ventilator strategies, ECLS use
• Version 2 2001-2006oUnderstanding the details - delivery, oxygen/carbon dioxide, discharge
status, cardiac anomalies
• Version 3 2007-2014oStaging - classifying defect size
• Version 4 2015-presentoThe role of the heart and PH, prenatal dx
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Issues addressed by version 4• Timing of surgical repair when receiving ECLS• Cardiac dysfunction in CDH• CDH-associated pulmonary hypertension• Prenatal diagnosis / prediction in CDH
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85 Centers/17 countries/12,000 Patients
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Hernia Side
Left Right
Bilateral
79% 20%
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Operative Approach
SubcostalThoracic
Other
90%6%
4%
1995-1996
Subcostal ThoracicOther
82%14%
1%
2017-2018
2%
1%ThoracoscopicLaparoscopic
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Patch Used In Repair
Yes No
49%51%
1995-1996
Yes
58% 42%
2017-2018
No
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Repair On ECMO (Of all ECMO)
Yes No
23% 77%
1995-1996
Yes No
52% 48%
2017-2018
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1200-1800
Time of Day For Repair
0600-12002400-0600
1800-2400
1995-1996
2%
10%40%
48%
1200-18001800-2400
0600-1200
2017-2018
45%
51%
4%
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05
101520253035404550
0-24 24-48 48-72 72-96 96-120 >120Hours of Age
Timing of Operation (No ECMO)
(38)
(14)(11)(10)
(8)
(19)
1995-1996
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0
50
100
150
200
250
0-24 24-48 48-72 72-96 96-120 >120Hours of Age
Timing of Operation (No ECMO)
(7)
(12)
(18)(18)
(13)
(32)
2017-2018
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Publications• Data available only to CDHSG members• Authored by writing committee on behalf of CDHSG• 55 publications • Multiple studies in progress
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0102030405060708090
100
Primaryrepair
Patch - noagenesis
Patch -Agenesis
Defect Size
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Size Does Matter!
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It became apparent that not all CDH were created equal and that size of defect was important
Version III designed to quantitate size of defect
Defect Size
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Standardized Reporting for Congenital Diaphragmatic Hernia
An International Consensus
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MethodsFactors Evaluated
Defect class Cardiac anomalies Chromosomal anomalies Birthweight /Gestational age Apgar Scores
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CDHSG Staging
A B C D
Lally, et al, J Pediatr Surg, 2013
44% 30% 13%13%96% 78% 58%99%Survival
Frequency
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V. 3 of Registry Grouped by defect size Compared for associated anomalies
Methods
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0
5
10
15
20
25
30
35
40
45
A B C D
All Major Anomalies Major Cardiac
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PEDIATRICS Volume 138: 2016:e2016204
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V. 3 of Registry Evaluated recorded morbidity at d/c Correlated degree of morbidity to defect Analysis between groups and time
Methods
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Defect correlated with morbidity as well as mortality Overall improving morbidity No major changes in large defect patients
Conclusions
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Is aggressive surgical management worth it?
Harting et al, Ann Surg, 2018
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Is aggressive surgical management worth it?
Harting et al, Ann Surg, 2018
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Aggressive approach leads to highest survival It is costly Morbidity is high
Conclusions
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What about Nitric Oxide ?
Putnam et al, JAMA Pediatrics, 2017
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What about Nitric Oxide ?
Putnam et al, JAMA Pediatrics, 2017
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What about Nitric Oxide ?
Putnam et al, JAMA Pediatrics, 2017
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Putnam et al, JAMA Pediatrics, 2017
What about Nitric Oxide ?
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Treatment with iNO was associated with a 15% higher absolute mortality
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iNO use highly variable between centers > 1/3 patients w/o CDH-PHTN received iNO Little data to support iNO benefit in CDH iNO use is associated with worse outcome iNO use in patients with CDH needs re-evaluation
Conclusions
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Centers with at least 22 years of continual participation Grouped by 5 year intervals Evaluated Overall Survival Looked at O:E survival
25 Years – Any Progress?
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Significant increase in survival over years Current overall survival is 73% for all comers Surgical survival is 85% Remains a large variation amongst centers
25 Years – Any Progress?
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The CDH Study Registry
• Ability to study infrequent problems• Data on very large number of patients• Individual centers can compare themselves with others• Demonstrate changes over time of management and outcome
PROs
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The CDH Study Registry
• Observational data• Inability to evaluate long-term sequelae• Difficult to collect complicated information• Wide spectrum of patients and treatment philosophies
CONs
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The “Gold Standard”Randomized Clinical Trial
• Expensive ($500k-$3 million+)• Labor intensive• Takes a long time (5-10 years)• Requires consent / challenges of recruitment• Requires multi-institutional cooperation• Answers a single question• Nearly impossible to achieve appropriate sample size in CDH
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The future of the CDHSG• Ongoing evolution of versions to address current questions
• Version 5 – Breakout session this meeting• Management standardization• Long term data collection• Novel statistical analysis
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John Roesler – DOB 11/26/2019
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