Disclosures. Not as Pink as You Think 3/17/2014. Not As Pink As You Think: Pulse Oximetry Screening For Critical Congenital Heart Disease

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1 March of Dimes New York State Chapter 36th Annual Perinatal Nurses Conference Promoting Perinatal Health Through Evidence Based Practice Not As Pink As You Think: Pulse Oximetry Screening For Critical Congenital Heart Disease Robert Koppel, MD March 24, 2014 Disclosures Dr. Koppel has nothing to disclose. Not as Pink as You Think 1

Think: Pulse Oximetry Screening For Critical Congenital Heart Disease Robert

2 Congenital Heart Disease 9/1,000 live births Approximately one quarter of these children will have critical congenital heart disease requiring surgery or catheter intervention in the first year of life Congenital Heart Disease Surgery allows for repair or palliation of nearly all congenital heart malformations Surgery and catheter interventions have resulted in a marked improvement in survival Surgery is often performed in the first weeks of life optimize hemodynamics prevent end organ injury timely diagnosis of CCHD can improve outcome for this population Window of opportunity for detection of critical congenital heart disease Newborn Nursery 2

catheter interventions have resulted in a marked improvement in survival Surgery is often performed in the first weeks of life optimize hemodynamics prevent end

3 Newborn Screening New York 1960s The Cyanotic Blind Spot Hokanson, Neonatology Today 2010;5(12):1-6. Morbidity Due to Delayed Diagnosis Shock global hypoxemic injury with multi organ dysfunction Hypotension Poor ventricular function Myocardial ischemia Pulmonary hypertension Renal dysfunction Hepatic dysfunction Decreased intestinal blood flow > NEC DIC Metabolic Hypoglycemia Hypocalcemia Myoglobinuria hypoxic ischemic encephalopathy 3

Poor ventricular function Myocardial ischemia Pulmonary hypertension Renal dysfunction Hepatic dysfunction

4 Fetal U/S Echocardiography fewer than 50% of cases are identified Best detection rate for single ventricle anatomy fewer than 30% detection of lesions with twoventricle anatomy Early Reports on Pulse Oximetry Screening Byrne, et al Identified in a population of 3896 consecutive asymptomatic newborns HLHS 1 Coarctation 1 Tetralogy of Fallot 1 Ped Res 1995, 37:198A Early Reports on Pulse Oximetry Screening Kao, et al: Using a cutoff of 95% 81% of infants with known critical congenital heart disease could be identified. Not effective at detecting aortic stenosis and coarctation Pediatr Res. 1995;37:216A 4

HLHS 1 Coarctation 1 Tetralogy of Fallot 1 Ped Res 1995, 37:198A Early Reports on Pulse Oximetry Screening Kao, et al: Using a cutoff of 95% 81% of

5 Method At time of dry blood spot collection, a reusable pulse oximeter probe is applied to the baby s foot Method When a steady wave form is displayed, the saturation is recorded Physician is notified for lower extremity saturation < 95% Pre and post ductal saturations are performed Echocardiogram is requested 5

saturation is recorded Physician is notified for lower extremity saturation <

6 LIJ Patient Diagnosis Detection 1 HLHS Fetal echocardiography 2 PA VSD Fetal echocardiography 3 Ebstein s Fetal echocardiography 4 HLHS Fetal echocardiography 5 PS Fetal echocardiography 6 ToF Fetal echocardiography 7 HLHS Fetal echocardiography 8 CoA Fetal echocardiography 9 IAA Fetal echocardiography 10 D TGA Cyanosis 11 Ebstein s Respiratory distress 12 D TGA Cyanosis 13 CoA Tachypnea/murmur 14 Truncus arteriosus Screening (SpO 2 86%) GSH Patient Diagnosis Detection 15 D TGA Cyanosis 16 PA Cyanosis 17 TAPVR Screening (SpO 2 92%) 18 TAPVR Screening (SpO 2 88%) Born at Hospitals Without Screening Patient Diagnosis Detection 19 CoA CHF, shock 20 HLHS Cyanosis, shock 21 ToF murmur False Negative Screen Patient Diagnosis Detection 22 CoA CHF 23 Hypoplastic LPA/AP collaterals Poor feeding, murmur 6

14 Truncus arteriosus Screening (SpO 2 86%) GSH Patient Diagnosis Detection 15 D TGA Cyanosis 16 PA Cyanosis 17 TAPVR Screening (SpO 2 92%) 18 TAPVR Screening (SpO 2 88%) Born at Hospitals Without

7 CCHD Hypothyroidism PKU Hearing Loss Frequency/ ,000 births # positive, first screen # children diagnosed PPV 75% 3% 80% 5% Cost of initial negligible $3 $3 $25 screen/child Screening cost/confirmed diagnosis negligible $10,800 $40,500 $9,860 Clinical result of delayed diagnosis Death CNS injury Cretinism Mental retardation Language, academic, and cognitive delays, psychosocial difficulties Circulation 2009;120; ; originally published online Jul 6, 2009 Pediatrics 2009;124; ; originally published online Jul 6, 2009 Clinical Studies of Pulse Oximetry Compilation of published studies Sensitivity 69.6% Specificity 99.9% Concerns about false positive rate and unnecessary echocardiograms After 24 hours, false positive rate 0.035% (~ 1/3000) 7

delays, psychosocial difficulties Circulation 2009;120;447-458; originally published online Jul 6, 2009 Pediatrics 2009;124;823-836; originally published online Jul 6, 2009 Clinical Studies of Pulse

8 Detection of CCHD Lesions (SpO 2 < 95%) CCHD Lesion Total Percent Detected DORV 3/3 100 HLHS 5/5 100 PA 5/5 100 d TGA 9/9 100 TAPVC 6/ Truncus 7/ TA 1/1 100 AA/AS 3/ TOF 9/ AVSD 4/ CoA 8/ PS 2/ Limitations of Oximetry Screening Less efficient at identifying left heart obstructive lesions limits the usefulness of oximetry as a screening tool Other types of lesions also result in morbidity/mortality All cases of HLHS were detected Coarctation detected in just over half of cases Limitations of Oximetry Screening Programmatic limitations Need for echocardiography Community hospitals Rural hospitals Solution Telemedicine 8

3 Limitations of Oximetry Screening Less efficient at identifying left heart obstructive lesions limits the usefulness of oximetry as a screening tool Other types of

9 CCHD 7 The seven defects classified as CCHD are: 1. Hypoplastic Left Heart Syndrome (HLHS) 2. Pulmonary Atresia with intact septum (PA/IVS) 3. Tetralogy of Fallot (TOF) 4. Total Anomalous Pulmonary Venous Return (TAPVR) 5. Transposition of the Great Arteries (TGA) 6. Tricuspid Atresia (TA) 7. Truncus Arteriosus communis (TAC) SACHDNC Summary Pediatrics August 22, 2011 A significant body of evidence suggests that early detection of CCHD through pulse oximetry screening is an effective strategy for reducing morbidity and mortality in young children. The workgroup identified strategies for hospitals and birthing centers to implement pulse oximetry screening 9

Truncus Arteriosus communis (TAC) SACHDNC Summary Pediatrics August 22, 2011 A significant body of evidence suggests that early detection of CCHD through pulse

10 SACHDNC Summary Pediatrics August 22, 2011 Screening should be conducted using motiontolerant pulse oximeters that report functional oxygen saturation cleared by the FDA for use in neonates SACHDNC Summary Pediatrics August 22, 2011 Screening in well baby and intermediate care nurseries should be based on the recommended screening algorithm, and be performed by any qualified health care personnel who have met training requirements (e.g., nurses, allied health technicians) SACHDNC Summary Pediatrics August 22, 2011 Any abnormal pulse oximetry screen requires a complete clinical evaluation by a licensed, independent practitioner. In the absence of other findings to explain hypoxemia, CCHD needs to be excluded, based on a comprehensive echocardiogram interpreted by a pediatric cardiologist before discharge to home. If an echocardiogram cannot be performed in the hospital or birthing center and diagnosis by telemedicine is not possible, strong consideration should be made for transfer to another medical center for diagnosis. Before implementing screening, protocols for arranging diagnostic follow up should be established. 10

11 AAP-AHA-CDC-HRSA The Lancet Volume 379, Issue , 30 June

12 Hospitalizations, Costs, and Mortality Florida ,128,236 live births in hospitals 3603 with CCHD 825 (23%) detected late 52% more hospital admissions 18% more hospital days 35% more inpatient costs Birth Defects Research (Part A): Clinical and Molecular Teratology DOI: /bdra Cost Effectiveness of Routine Screening for CCHD in US Newborns Mathematical models based on Florida more newborns with timely CCHD detection 20 deaths averted by screening 1975 false positive results minimal impact on total estimated program costs Additional cost of $6.28 per newborn $20,862 per newborn with CCHD detected by screening $40,385 per life year gained per newborn with CCHD detected by screening Pediatrics 2013;132:1-9 CCHD < 28 days ED/PICU Transport no screening at birth hospital CCMC Coarctation 10 Tetralogy of Fallot 3 TAPVR 2 Interrupted arch 2 HLHS 2 Aortic stenosis 1 Pulmonary atresia 1 Pulmonary stenosis 1 Tricuspid atresia 1 Ebstein s anomaly 1 12

23165 Cost Effectiveness of Routine Screening for CCHD in US Newborns Mathematical models based on Florida 1998 2007 1189 more newborns with timely CCHD detection 20 deaths averted by screening 1975

13 Case Presentation 40 weeks gestation, C/S, 3600 grams Discharged home on day 3 Day 5: returned to ED for poor feeding and decreased activity SpO 2 : 80% ABG: ph 6.8 Echo: HLHS Pre op stabilization X 5 days Norwood stage I Post op ECMO X 8 days Case Presentation 39 weeks, NSVD, Apgar 9/9 Discharged home on Day 2 Oximetry screening post ductal SpO2 100% Day 3 Lethargy Decreased PO intake Dry diapers Tachypnea Evaluated by pediatrician 13

8 Echo: HLHS Pre op stabilization X 5 days Norwood stage I Post op ECMO X 8 days 2012 2014 www.cchdscreeningmap.

14 Case Presentation Referral to ED for respiratory distress grunting Retracting unable to measure SpO2 Intubated Umbilical arterial and venous catheters inserted Case Presentation ABG: 7.09/17/199/8/ 23.3 Chemistry: 143/8/104/6/63/5.98 Echo: coarctation, DA closed (history of normal fetal echo) Prostaglandin infusion Dialysis prior to CoA repair Swiss Cheese Model of Accident Causation British Medical Journal 320 (7237):

15 Swiss Cheese Model of CCHD Screening Failure Swiss Cheese Model of Accident Causation Obstetric ultrasound Fetal echo Newborn physical exam Nursery course Oximetry screening British Medical Journal 320 (7237): Technical Factors False positive and negative readings Poor perfusion Motion artifact Ambient light Partial probe detachment Differences between manufacturers Pediatr Cardiol (2008) 29:

320 (7237): 768 770 Technical Factors False positive and negative readings Poor perfusion Motion

16 Human Factors Phase I fewer than half of readings reliable Phase II 60% of readings reliable Improved reliability correlated with Higher level nursing degree LPN or higher Amount of time spent > 360 seconds Pediatr Cardiol (2008) 29: Accuracy: Paper Algorithm v. Computer Based Tool J Pediatr Jan;164(1):67-71.e2. doi: /j.jpeds Ease of Use: Paper Algorithm v. Computer Based Tool J Pediatr Jan;164(1):67-71.e2. doi: /j.jpeds

29:885-889 Accuracy: Paper Algorithm v. Computer Based Tool J Pediatr. 2014 Jan;164(1):67-71.e2. doi: 10.1016/j.jpeds.

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18 Helpful Hints Neonatal probe specific to oximeter Do not use adult probe Follow manufacturer s instructions Clean probe (re usable or single use) Apply to clean skin Best sites: palm and foot Light source and sensor directly opposite Preferably awake, calm, and quiet baby Avoid extraneous light Ideal CCHD Screening Program Education Nurses Normal newborn NICU Formal screener training Competency Continuing education Physicians Pediatricians Neonatologists Cardiologists Continuing education 18

awake, calm, and quiet baby Avoid extraneous light Ideal CCHD Screening Program Education Nurses Normal newborn NICU Formal

19 Ideal CCHD Screening Program Education Parents Negative screen Positive screen languages Literacy Ideal CCHD Screening Program Standardization of algorithm Minimize variation Equipment Supplies Ideal CCHD Screening Program Universal testing Quality of Process Database Local Public health 19

of algorithm Minimize variation Equipment Supplies Ideal CCHD Screening

20 Ideal CCHD Screening Program Documentation Automatic link to EHR Automatic reporting to DOH Ideal CCHD Screening Program Communication Parents Pediatrician Cardiologist 20

Screening for Critical Congenital Heart Disease in the Apparently Healthy Newborn

Screening for Critical Congenital Heart Disease in the Apparently Healthy Newborn A presentation of Texas Pulse Oximetry Project: A Joint Educational Initiative of The University of Texas Health Science