A2a. Fundamentals of CDH Care. Session Summary. Session Objectives. References

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1 A2a Fundamentals of CDH Care David Kays, MD Professor of Surgery Johns Hopkins School of Medicine Director of Congenital Diaphragmatic Hernia Program, Director of Extra Corporeal Life Support Program Johns Hopkins All Children s Hospital, St. Petersburg, FL The speaker has signed a disclosure form and indicated he has no significant financial interest or relationship with the companies or the manufacturer(s) of any commercial product and/or service that will be discussed as part of this presentation. Session Summary Optimizing survival in CDH infants requires neither finding a new drug nor developing a new mode of ventilation, but rather lies in understanding CDH physiology and adhering to the fundamentals of CDH care. This talk, based on CDH survival rates thought unobtainable, will outline those fundamentals. Session Objectives At the conclusion of this activity, participants will be able to: describe the anatomic and physiologic markers that relate to CDH severity; discuss the role of strict lung protective ventilation in CDH; state the importance of CDH repair in CDH survival outcomes. References Balayla, J. & Abenhaim, H. A. (2014). Incidence, predictors and outcomes of congenital diaphragmatic hernia: A population-based study of 32 million births in the United States. The Journal of Maternal-Fetal & Neonatal Medicine: the Official Journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians, 27(14), Kays, D. W., Talbert, J. L., Islam, S., Larson, S. D., Taylor, J. A. & Perkins, J. (2016). Improved survival in left liverup congenital diaphragmatic hernia by early repair before extracorporeal membrane oxygenation: optimization of patient selection by multivariate risk modeling. Journal of the American College of Surgeons, 222(4), Kays, D. W., Islam, S., Larson, S. D., Perkins, J. M. & Talbert, J. L. (2013). Long-term maturation of congenital diaphragmatic hernia treatment results: Toward development of a severity-specific treatment algorithm. Annals of Surgery, 258(4), , discussion Kays, D. W., Islam, S., Perkins, J. M., Larson, S. D., Taylor, J. A. & Talbert, J. L. (2015). Outcomes in the physiologically most severe congenital diaphragmatic hernia (CDH) patients: Whom should we treat? Journal of Pediatric Surgery, 50(6), Kays, D. W., Islam, S., Richards, D. S., Larson, S. D., Perkins, J. M. & Talbert, J. L. (2014). Extracorporeal life support in patients with congenital diaphragmatic hernia: How long should we treat? Journal of the American College of Surgeons, 218(4), Kays, D. W., Langham, M. R., Ledbetter, D. J. & Talbert, J. L (1999). Detrimental effects of standard medical therapy in congenital diaphragmatic hernia. Annals of Surgery, 230(3), 340-8, discussion A2a: Fundamentals of CDH Care Page 1 of 11

2 Kays, D. W., Talbert, J. L., Islam, S., Larson, S. D., Taylor, J. A. & Perkins, J. (2016). Improved survival in left liver-up congenital diaphragmatic hernia by early repair before extracorporeal membrane oxygenation: optimization of patient selection by multivariate risk modeling. Journal of the American College of Surgeons, 222(4), Seetharamaiah, R., et al. (2009). Factors associated with survival in infants with congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation: A report from the Congenital Diaphragmatic Hernia Study Group. Journal of Pediatric Surgery, 44, A2a: Fundamentals of CDH Care Page 2 of 11

Hopkins All Children s Hospital October 18 th 2016 Why should you listen to me?

3 Fundamentals of CDH Care: Part Faculty at Johns Hopkins University School of Medicine Johns Hopkins All Children s Hospital St. Petersburg, Florida David W. Kays M.D. FACS FAAP Professor of Surgery Johns Hopkins University School of Medicine Director of Congenital Diaphragmatic Hernia Program Director of Extracorporeal Life Support Program Johns Hopkins All Children s Hospital October 18 th 2016 Why should you listen to me? CDH is Important (1) Cared for > 325 CDH No patients turned away Overall Survival (everyone) = 80% Survival of isolated CDH now > 95%. ALL pts d/c breathing on their own. No patients d/c with tracheostomy. None CDH affects 1 in every 3000 live births In 2014 in the USA, there were 3,988,076 births 800 with gastroschisis and fewer than 100 died (*estimate) 500 will develop Wilms tumor, and fewer than 100 died or will die > 1300 fetuses will have CDH. 650 or more will die from CDH, from termination or during treatment CDH is Important (2) CDH is a Frequently Lethal Defect CDH Patients are Severity Outliers: CDH patients suffer from Small lungs and pulmonary hypertension Under developed hearts Are at risk of hypoxic ischemic brain injury Need exacting critical care Pulmonary Hypoplasia Pulmonary Hypertension Fixed component due to small lungs Reactive component due to CDH physiology time course Advancing the care of CDH babies improves the care of all neonates A2a: Fundamentals of CDH Care Page 3 of 11

Baylayla et al, J Mat Fetal & Neo Medicine 2014 CDH

Liver down (least severe) Right: more severe (on

(2009); CDH Study Group Seetharamaiah et al, J Ped

stomach down) Prognosis is excellent L CDH, Liver

small) Intestine in chest Stomach in chest L CDH:

liver-up R lung is also very small, poorly inflated

4 What is the Current CDH Survival? 54% Survival (2014) (Canadian Population Study) Baylayla et al, J Mat Fetal & Neo Medicine 2014 CDH is a Spectrum Can define by Herniated Viscera Left: Liver down (least severe) Right: more severe (on average) Left: Liver Up (most severe) 68% Survival (2009); CDH Study Group Seetharamaiah et al, J Ped Surgery, 2009 L CDH, Liver-down (Least severe, stomach down) Prognosis is excellent L CDH, Liver Down (Stomach Up) Heart in R chest Left lung (v. small) Intestine in chest Stomach in chest L CDH: Most Severe (Liver Up) Extremely Severe CDH Large liver-up R lung is also very small, poorly inflated ABG s are dismal (remember this child) First ABG: 6.64 / >130 / 15 A2a: Fundamentals of CDH Care Page 4 of 11

CDH is a Spectrum CDH is a Spectrum Can define by Lung Size: Lung to head ratio (LHR). 3-D Lung volumes LHR > 1.4 less severe LHR 1.0 1.4 more severe LHR < 1.

org Blood gas values at 1 hour of life (ph, PCO2, PO2) Defining CDH Severity by Physiology Survival by Physiologic variables at birth and soon after CDH Severity by Anatomic Grading Data from

5 CDH is a Spectrum CDH is a Spectrum Can define by Lung Size: Lung to head ratio (LHR). 3-D Lung volumes LHR > 1.4 less severe LHR more severe LHR < 1.0 most severe Can define by physiology at birth and soon after Apgar scores at 1 and 5 minutes Birth weight CDH Study Group predicted survival (birth weight and 5 minute Apgar) Nicutools.org Blood gas values at 1 hour of life (ph, PCO2, PO2) Defining CDH Severity by Physiology Survival by Physiologic variables at birth and soon after CDH Severity by Anatomic Grading Data from n= 240 CDH Left Liver Down Right Left Liver Up N=97 N=42 N=101 Birthweight / / / 683 Apgar / / / 2.2 Apgar / / / 2.2 ECMO 11% 55% 69% CDH SG Predicted Survival 74% 62% 53% And what about Left Liver-Up CDH? - Most severe anatomic subset - Worst pulmonary hypoplasia - 45% Survival 2010 Meta-analysis survival - 45% Survival 2012 Top CDH Children s Hospital Publication Mullassery D, et al, Ultrasound Obstet Gynecol. 2010;35: Victoria T, et al. Prenat. Diagn.; 2012;32: Most CDH are diagnosed prenatally: What do we tell expectant parents? (1) Major Associated Anomalies (10% of total) Cardiac: Uni-ventricular heart (poor prognosis) Fetal echo Genetic: Major chromosome and Syndromes (poor prognosis) Amniocentesis CNS (poor prognosis) (2) CDH Severity - Volume of herniated viscera - Lung size - Physiology at birth A2a: Fundamentals of CDH Care Page 5 of 11

Fundamentals of CDH Care CDH: Treatment of Surgery 23 Years of CDH care and Research (320 CDH patients) 5 major lessons learned Lungs (ventilation must be lung protective.

result in improved survival Prospective change in therapy in August, 1992 Annals of Surgery. 1999.

comfort and clinical state Lowest pressure which provides adequate chest movement (usually 20 24 cm H2O) PIP > 25 cm H2O used uncommonly, and usually as bridge to ECMO (Mean airway pressure <12 cm

6 Fundamentals of CDH Care CDH: Treatment of Surgery 23 Years of CDH care and Research (320 CDH patients) 5 major lessons learned Lungs (ventilation must be lung protective. Really) Repair (required) ECMO (Not all ECMO is the same) Risk stratification to define care Believe Hypothesis: Hyperventilation/alkalosis is harmful to CDH patients Elimination of this therapy will result in improved survival Prospective change in therapy in August, 1992 Annals of Surgery (3) Kays, Langham, Ledbetter, and Talbert CDH: Treatment of Surgery Light to moderate sedation (no paralysis) Conventional SIMV pressure limited ventilation with rate set to patient comfort and clinical state Lowest pressure which provides adequate chest movement (usually cm H2O) PIP > 25 cm H2O used uncommonly, and usually as bridge to ECMO (Mean airway pressure <12 cm H2O) Hyperventilation and alkalosis are strictly avoided Ventilation/Oxygenation Goals Goal PaCO2: Will Tolerate: Goal PaO2: Will Tolerate: Much lower IF: Pre-ductal sats> 85% and stable or rising Perfusion is satisfactory Serum lactate is normal and not rising Goal ph:> 7.20 Treat with NaHCO3 Annals of Surgery (3) Keep PIP minimal. Preserve lung parenchyma Mean PIP over 120 hours Survival Curve by Era, All Patients Mean +/- SEM p<0.05 at all time points p= Time*Era effect Survival Graph p< Annals of Surgery (3) Annals of Surgery (3) A2a: Fundamentals of CDH Care Page 6 of 11

Mean PIP over 120 hours Mean PIP over 120 hours Mean +/- SEM Mean +/- SEM p<0.05 at all time points p<0.05 at all time points p=0.00001 Time*Era effect p=0.

7 Mean PIP over 120 hours Mean PIP over 120 hours Mean +/- SEM Mean +/- SEM p<0.05 at all time points p<0.05 at all time points p= Time*Era effect p= Time*Era effect PaCO2 over 120 hours by Era Arterial ph over 120 hours Mean +/- SEM Mean +/- SEM p< 0.05 at all time points except T= 12 and 108 hours p< 0.05 at all time points except t=12 hours p<0.05 Time*Era effect p< Time*Era effect Survival Curve by Era, All Patients CDH Treatment Fundamental #1 Survival Graph p< Must eliminate any iatrogenic lung injury: The number of CDH patients that survive is all about how well we take care of their lungs A2a: Fundamentals of CDH Care Page 7 of 11

8 CDH Referral Pattern Repair of CDH: What matters? Open? Thoracoscopic? Patch? Which patch? UF to Johns Hopkins All Children s, St. Petersburg, FL High volume Referral Center High percentage of prenatally diagnosed and evaluated patients Expanding US and International Referrals 32 Very large Goretex patch repair Repair: Liver in chest A small lung A2a: Fundamentals of CDH Care Page 8 of 11

A very small lung CDH Treatment Fundamental #2 (2) Repair the Hernia (CDH) (n=268) 1 CDH Survival to Discharge 0.9 0.8 0.7 0.6 0.5 0.4 0.3 0.2 0.

Timing of Repair Severe Associated Anomalies: (10%) - Chromosomal - Cardiac (single ventricle anatomy) - CNS Repair was delayed: never got repaired - Repair was delayed for stabilization or

9 A very small lung CDH Treatment Fundamental #2 (2) Repair the Hernia (CDH) (n=268) 1 CDH Survival to Discharge Repaired Not Repaired Why wouldn t a CDH be repaired? Timing of Repair Severe Associated Anomalies: (10%) - Chromosomal - Cardiac (single ventricle anatomy) - CNS Repair was delayed: never got repaired - Repair was delayed for stabilization or resolution of P. HTN Ended up on ECMO unrepaired ECMO course went poorly (complications) - These patients are potentially recoverable N = 268 Evaluated surgical repair timing related to ECMO Results stratified by anatomic severity Annals of Surgery, 2013 Kays, Islam, Larson, Perkins, Talbert Fundamentals of CDH Care ECMO: Role in CDH 23 Years of CDH care and Research (320 CDH patients) 5 major lessons learned -Lungs (Ventilation must be lung protective. Really!) -Repair (required) -ECMO (Not all ECMO is the same) -Risk stratification to define care -Believe Centers with highest reported survival use ECMO ECMO use averages 25 50% of patient Evaluated Surgical repair timing related to ECMO Results stratified by anatomic severity A2a: Fundamentals of CDH Care Page 9 of 11

10 CDH Quantity of Survival Care of lungs Putting a face with the numbers L Liver Up CDH Diagnosed prenatally at 14 weeks LHR 0.69 At birth, on VA ECMO w/in 4 hours Repaired on ECMO at 3 days 2 ECMO runs lasting 29 days Quality of Survival Care of brain NP Focused Protocols Neuro-protective Brain monitoring NIRS Biomarkers of brain injury Correlation with Outcomes Much more work to do: Passionate people and families Improved Risk Stratification to refine repair timing decisions Development of more specific protocols: Neuro protection Expanded education Expanded collaborations Defining Neurologic outcomes MRI findings at discharge Robust neurodevelopmental f/u Feeding and growth outcomes balanced with pulmonary function Reported by risk stratification Critical Evaluation of ECMO type Effect of prematurity Long term Pulmonary HTN outcomes more A2a: Fundamentals of CDH Care Page 10 of 11

Thank You: Johns Hopkins All Children s Hospital Johns Hopkins School of Medicine University of Florida Research and Program Coordinator Joy Perkins, RN, RRT Partners and Mentors Paul Colombani, MD

11 Thank You: Johns Hopkins All Children s Hospital Johns Hopkins School of Medicine University of Florida Research and Program Coordinator Joy Perkins, RN, RRT Partners and Mentors Paul Colombani, MD Jonathan Ellen, MD Saleem Islam, MD Nicole Chandler, MD David Burchfield, MD Max R Langham, MD Jen T. Wung, MD Paul Danielson, MD James L. Talbert, MD Shawn Larson, MD Janice Taylor, MD Mike Weiss, MD Charles Stolar, MD Johns Hopkins All Children s Hospital Pediatric Surgery Fellows Dan Neal, Statistician A2a: Fundamentals of CDH Care Page 11 of 11

A2b. Risk Stratification of CDH Repair Timing and ECMO Support of CDH. Session Summary. Session Objectives. References

A2b. Risk Stratification of CDH Repair Timing and ECMO Support of CDH. Session Summary. Session Objectives. References A2b Risk Stratification of CDH Repair Timing and ECMO Support of CDH David Kays, MD Professor of Surgery Johns Hopkins School of Medicine Director of Congenital Diaphragmatic Hernia Program, Director of