16th Asia-Pacific Congress of Pediatrics

26-29 Aug 2018 Bali Indonesia

Symposium 8: 29 Aug 2018 1200-1330
Pediatric Cardiac Emergency in Children: What Pediatricians Should Know?

Pulse Oximetry Screening

of the Newborn for
Cyanotic Congenital Heart Disease
Hung Liang Choo
Consultant Paediatrician & Paediatric Cardiologist
Hospital Kuala Lumpur

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 Pulse Oximetry Screening of the Newborn for
Cyanotic Congenital Heart Disease
What Pediatricians Should Know?

OUTLINE
• Introduction

• Evidence for Newborn Screening for Congenital Heart Disease

• Physical examination
• Pulse oximeter checks
• Physical examination & pulse oximetry checks

• Strategies for Implementation

• Barriers and Challenges

• Conclusion
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INTRODUCTION

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Congenital Heart Disease (CHD)
• Anatomic malformation of the heart ± its vessels which occurs during intrauterine life
• Clinical Classification: acyanotic, cyanotic
Acyanotic CHD
Shunt lesion
• Ventricular septal defect
• Patent ductus arteriosus
• Atrial septal defect
• Atrioventricular septal defect
• AP window
• Coronary artery fistula
Obstructive lesion
 Pulmonary stenosis
 Coarctation of the aorta
 Aortic stenosis
Cyanotic CHD
Conotruncal anomalies
• Tetralogy of Fallot
• Transposition of the great arteries
• Pulmonary Atresia ± VSD
• Truncus arteriosus
• Double outlet RV / LV
• Interrupted aortic arch (Type B)
Others
• Tricuspid atresia & Single ventricle
• Hypoplastic left heart syndrome
• Total anomalous pulm venous drainage
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 Definition of Echo Findings
DEFINITION ECHO FINDINGS

Normal No echocardiographic abnormalities

Non-significant No clinical signs

Small PDA, small PFO/ASD, musc VSD, turbulence branch PAs
Not detected after 6 months

Significant Small PDA, small PFO/ASD, musc VSD, turbulence branch PAs
Findings persist for longer than 6 months
Any cardiac lesion requires regular monitoring after 6 months or drug treatment, but is
not classified as serious or critical

Serious Any cardiac lesions that is not defined as critical, but requires intervention (cardiac
cath or surgery) within 1 year of age

Critical Duct-dependent lesions: HLHS, PAIVS, simple TGA, IAA.

All infants dying or requiring surgery within 28 days of life
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AK Ewer. Lancet 2011;378:785-94
– CoA, AS, PS, TOF, PA-VSD, TAPVD.
What are Critical Congenital Heart Disease?

• All infants with duct-dependent lesions:

• Hypoplastic left heart syndrome (HLHS)
• Pulmonary atresia with intact ventricular septum (PAIVS)
• Simple transposition of the great arteries (TGA)
• Interruption of the aortic arch (IAA)
• All infants dying or requiring surgery within the first 28 days of life
• coarctation of the aorta
• aortic valve stenosis
• pulmonary valve stenosis
• tetralogy of Fallot
• pulmonary atresia with ventricular septal defect
• total anomalous pulmonary venous connection
AK Ewer. Lancet 2011;378:785-94 6
 Critical Heart Lesions

Obstructed Total Anomalous

Duct-dependent parallel circulation
Pulmonary Venous Drainage
• Simple TGA
(TAPVD)

• Duct-dependent pulmonary circulation

• Obstructed antegrade pulmonary flow
• PA IVS
• Critical PS
• Tricuspid atresia w restrictive VSD / PS
• Pulmonary atresia + VSD
• Pulmonary atresia + Complex CHD
• Truncus arteriosus
• Duct-dependent systemic circulation
• Severely obstructed aortic flow
• Hypoplastic left heart syndrome
• Interrupted aortic arch
• Severe Coarctation of Aorta
• Critical Aortic Stenosis
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 Pulse Oximetry

To detect low blood oxygen saturation

CHD lesions targeted are those with hypoxemia /desaturation SaO2 <95%

1. Require intervention in the first year of life

2. Present of hypoxemia some or most of the time

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 Clinical Classification of CCVMs in Children
Acyanotic CHD
Shunt lesion
• Ventricular septal defect
• Patent ductus arteriosus
• Atrial septal defect
• Atrioventricular septal defect
• AP window
• Coronary artery fistula
Obstructive lesion
 Pulmonary stenosis
 Coarctation of the aorta
 Aortic stenosis
Cyanotic CHD
 Conotruncal anomalies
• Tetralogy of Fallot
• Transposition of the great arteries
• Pulmonary Atresia ± VSD
• Truncus arteriosus
• Double outlet RV / LV
• Interrupted aortic arch (Type B)
• Others
• Tricuspid atresia & Single ventricle
• Hypoplastic left heart syndrome
• Total anomalous pulm venous drainage
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Evidence for Pulse Oximetry
Screening of Newborn for
Congenital Heart Disease

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 Newborn Screening
• First started by Dr. Robert Guthrie in 1960s for phenylketonuria (PKU)
• Essential, preventive public health program for early identification of
disorders in newborns that can effect their long term health
• Expanded …..
• Congenital hypothyroidism
• Galactosemia
• Sickle cell disease
• G6PD deficiency
• Metabolic disorders
• Hearing
• CCHD ……
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Wilson’s (Jungner) criteria for newborn screening 1968

 Clinically and biochemically well-defined disorder

 Known incidence in the population
 Disorder associated with significant morbidity and mortality
 Effective treatment available
 Period before onset, during which intervention improves outcome
 Ethical, safe, simple and robust screening test
 Cost effectiveness of screening

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 Congenital Heart Disease (CHD)
Burden of Disease
Incidence In Indonesia:
• 8 - 12 per 1000 live births (birth prevalence) ~ 5 million live births per year
~ 500,000 with CHD
• Asia 9.3 per 1000 live births
• More pulmonary outflow obstruction  ~ 100 000 CCHD
• Critical CHD
• 15% - 25% of all CHD

• 1 in 10 stillborns
• significant cardiac anomaly

• 20 - 75 per 1000 population

Hoffman JI et al. J Am Coll Cardiol. 2002 Jun 19;39(12):1890-900

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Van der Linde D et al. J Am Coll Cardiol.2011 Nov 15;58(21):2241-7
 Why Screen?

• Leading cause of death during infancy

• 30% of all infant fatalities in US annually

• Treatment available
• CCHD are treatable conditions
• Outcomes acceptable

Mahle WT. Pediatrics.2009;124:823-836

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Consequences of Late Detection

1. In the first few days of life

• 30% -50% of CCHD are asymptomatic & appear normal on physical examination
2. Risk of mortality is 30% if not diagnosed during birth hospitalization
• Die at home
• Present in poor clinical state  death
• Misdiagnosed as pneumonia or sepsis

3. Evidence that delay in diagnosis and treatment is associated w

• More hospitalization days
• increased cardiovascular compromise
• end-organ dysfunction
• high mortality
• Poor neurodevelopmental outcome after successful intervention
• Greater number of admissions
• Higher in-patient costs
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Why Screen? – Benefits of Screening

• Timely identification of infants prior to discharge from birth

hospitalization

• Minimize morbidity and mortality associated with delayed diagnosis

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 Newborn Screening for CHD

. Antenatal ultrasound
Fetal echocardiography
Newborn Screening
1. Postnatal clinical examination

2. Newborn pulse oximetry screening

3. Pulse oximetry & clinical exam of newborn

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 Antenatal Screening for Fetal Cardiac Abnormalities
• Introduced since 1980s
• Overall detection rate remains LOW
• 43.6% Scholler 2011
• ONLY Experienced tertiary centers
report high level of diagnostic
accuracy
Limitations
• Limited number of trained sonographer
• Operator dependent
• More likely to detect uni-ventricular heart
• Lesions that do not affect appearance of 4-chambers
 TGA, TAPVD, CoA likely to be missed
• Increased scheduled delivery
• Time consuming / multiple scans
• Expensive equipment
• Low detection rate
Unsuitable as screening tool
Newborn Physical Examination

• Cyanosis

• Femoral pulse

• Heart murmur

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Newborn Physical Examination –
fails to detect Critical Heart Lesions
• Cyanosis
• In newborns with normal Hb, cyanosis is only visible when SpO2 < 80%
• Many critical CHDs have SpO2 80-95%
• Skin pigmentation

• Pulses
• Femoral pulses may be normal when PDA is big

• Heart Murmur
• Absence of murmurs in 50% CHDs, mostly CCHD
• TGA, HLHS, IAA, CoA, TAPVD

• Flow murmurs are common during newborn period

• Closing PDA
• Transient TR
• Peripheral PA stenosis

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Newborn Physical Examination fails to detect Critical
Heart Lesions
In UK, studies suggested ~ 25% of infants with CCHD
were not diagnosed until after discharge1

Poor performance as screening test2

Sensitivity 44% (CI 31 – 51%)
Positive predictive value 54% (CI 39 – 69%)

Baltimore Washington Infant Study 1981 – 19893

4390 cases of Congenital heart disease
 800 (18.2%) deaths,
 76 (9.5%) deaths before diagnosis

1. Wren C, Richmond S, Donaldson I. Presentation of CHD in Infancy: implication for routine examination. Arch Dis Child Fetal Neonatal Ed 1999;80:F49-53
2. Ainsworth SB. Prevalence & clinical sig of cardiac murmurs in neonates. Arch Dis Child Fetal Neonatal Ed. 1999;80: F43-45
3. Pediatrics 1999 Apr; 103 (4 Pt 1) : 743 – 7 21
 Pulse Oximetry Screening for Critical CHD –
many barriers before 2007
• Extensively studied, many barriers before 2007
• Methodology variations
• Difficulties in assessment of accuracy
• Type of oximeters
• Timing of measurement
• Cutoffs for positive results

• Patient selection
• Types of CHD
• Sample size
• Follow-up

• Detection 8% by physical exam Vs 28% pulse oximeter

• Death 5 Vs 0
• A W Granelli. BMJ 2009;338:a3037

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AW Granelli. BMJ 2009;338:a3037

AK Ewer. Lancet 2011;378:785-94

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Pulse Oximetry Screening for Critical CHD in Newborn Infants
(2011)
• Pulse oximetry is
• Safe
• Non-invasive
• Feasible
• Reasonably accurate test
• Sensitivity better than antenatal screening or clinical examination
• Use of both preductal & postductal saturations compared with postductal
saturation alone seems advantages and did not take much longer to do.
• Detection of other diseases
• Strong evidence the potential benefits of introduction of pre-discharge
pulse oximetry screening as a routine procedure

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 AHA Fact Sheet 2013

In USA, almost all states require mandatory newborn screening for CCHD
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Objective
• To determine the diagnostic accuracy of pulse
oximetry as a screening method for detection of
CCHD in asymptomatic newborn infants

Main results
Search till March 2017 – 21 studies.
19 studies (436 758 participants) provided data for
primary analysis using SaO2 threshold <95% or ≤ 95%

Overall detection of CCHD

Sensitivity 76.3% (95% CI 69.5 – 82.0)
Specificity 99.9% (95% CI 99.9 - 99.9)
False positive-rate 0.14% (95% CI 0.07 – 0.22)

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27
CONCLUSION

• Pulse oximetry is a highly specific and moderately

sensitive test for detection of CCHD with
very low false-positive rate

• Current evidence supports the introduction of routine

screening for CCHD in asymptomatic newborn
before discharge from well-baby nursery

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 Strategies for Implementing Pulse Oximetry Screening
• Resources
• Human
• Equipment

• Process – standardized approach

• Protocol
• Criteria / Standards

• Diagnostic Follow-up

• Outcome & Audit

• Continuous quality improvement
• Track and trend

• National program
• Implementation & Surveillance
• Public Health Agency
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Kemper AR et al. Pediatrics 128(5),2011
Introduction of Pulse Ox in Malaysia (2011-12)
• Nation wide survey
• manpower
• pulse oximeter in postnatal or neonatal wards

• Proposal to the Ministry of Health for Pulse Oximeter and Manpower

to implement pulse oximetry Screening of Newborn for CCHD
• Local Protocol or algorithm
• Implementation of screening
• Audit, Quality Improvement, track and trend
• Oral presentation - Perinatal Congress Malaysia 2013. Study period: 16th Jan 2013 - 15th Apr 2013
• Poster presentation. 15th ASEAN Pediatric Federation Congress 2014

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Pulse Oximeter to Screen for Critical CHD
• Which pulse oximeter
• Motion tolerant sensors which function in states of low
perfusion

• Radical-7 pulse oximeter, reusable probe (Masimo)1

• low intra- & interobserver variability
• Accurate stable saturations in active babies, low
perfusion states

• Appropriate to use either disposable or reusable probes

In IPHKL, HO will take 1 pulse oximeter from neonatal ward to perform screening
1. AK Ewer. Lancet 2011;378:785-94
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STUDY DESIGN for Pulse-Ox

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AK Ewer. Lancet 2011;378:785-94
 33
Kemper AR et al. Pediatrics 128(5),2011
 34
Kemper AR et al. Pediatrics 128(5),2011
Systematic Review & Meta-analysis

S Thangaratinam. Lancet 2012

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 Sensitivity & Specificity

Sensitivity Specificity False positive

Study Method (%) (%) rate (%)
Pulse Ox 77.8 99.9 0.1
Riede et al1 Foot

Pulse Ox 75 99.1 0.84

Ewer et al2 RH & Foot

Clinical exam & 83.6 99.7 0.3

Zhao et al3 Pulse Ox

1. Riede FT. Effectiveness of neonatal pulse oximetry screening for detection of critical congenital heart disease in daily clinical routine—
results from a prospective multicenter study. Eur J Pediatr 2010.
2. Andrew K Ewer et al. on behalf of the PulseOx Study Group. Pulse oximetry screening for congenital heart defects in newborn infants
(PulseOx): a test accuracy study. Lancet 2011.
3.Qu-ming Zhao et al. Pulse oximetry with clinical assessment to screen for congenital heart disease in neonates in China: a prospective
study. Lancet 2014. 36
Pulse Oximetry Screen for Critical CHD – How?

• Probe/sensor site
• Foot only (post-ductal) vs
• Right hand & foot (pre- & post-ductal)
• Simultaneous (need 2 pulse oximeters)
• sequentially

• What SpO2 cut off threshold?

• > 95% - normal
• < 95% - repeat, inform doctor
• <90% - immediate evaluation

• ≤ 2% / ≤ 3% absolute difference between upper & lower extremities consider a pass

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Protocol for Newborn Pulse Oximetry Screening for the Detection of Congenital Heart Disease HKL

Newborn participant eligible for screening: A dedicated House Officer doing routine newborn checks in post-
•Place the mother’s sticker ID onto the log book natal ward will place the Masimo Medical-7 pulse oximeter probe
•Document baby’s: over any of the lower limb extremities of the baby & wait until a
- Gestation, Birth Wt, Gender consistent reading and good pulse waveform signal are displayed.
- Age (hours of life)
oDate & time of birth
oDate & time of test
Pulse Oxymeter reading < 95%
*test should be perform before discharge, (>6 hours of life).

 Warm up the baby’s feet

Pulse Oxymeter reading ≥ 95%  Repeat the measurement 1 hour
after the first test

Negative (PASS) If Pulse Oximeter reading < 95% twice (FAIL):

* Result will be documented into a log book and  Medical Officer in-charge of postnatal wards attends to baby
baby’s home base card immediately, perform full physical examination and
 admit the baby to SCN for close observation.
 Identify cause of hypoxemia.
 ECHO if indicated
 Inform case to specialist
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Pulse Oximetry Screen for Critical CHD – How?
• When to do pulse oximetry?
• After 6 hours of life and just before discharge

 Which pulse oximeter

• Motion tolerant sensors which function in states of low perfusion

 Probe/sensor site
• Foot only (post-ductal) vs
• Both right hand & foot (pre- & post-ductal)

 What SpO2 cut off threshold?

• > 95% - normal
• < 95% - inform doctor
Pulse Oximetry Screen for Critical CHD – when to check
• When to do pulse oximetry?

• Before 24 hours of age or at discharge

AK Ewer. Lancet 2011;378:785-94

• No earlier than 24 hours, prior to discharge from birthing

center AAP Policy Statement. Pediatrics 2012;129(1):190-192

• Between 6 hours and 72 hours

Qu-ming Zhao Lancet 2014;384:747-54

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Many babies are discharge < 24 hours

median age 12.4 hours

• Early testing and use of more

conservative cutoff thresholds
 Increases rate of
identification of babies with
disease
 At the expense of slight
increase false positive rate

AK Ewer. Lancet 2011;378:785-94 41

 Total livebirth: 6216

Admitted: 1538 (24.7%) Not admitted: 4678 (75.3%)

Cardiac cases: Non-cardiac cases: Screened: Not-screened:

14 (0.91%) 1524 (99.09%) 4151(88.73%) 527(11.27%)

Antenatally Antenatally positive: negative: Cardiac:

Non-cardiac:
Diagnosed: not diagnosed: 6 (0.14%) 4145 (99.86%) 2(0.38%)
525(99.62%)
4 (28.57%) 10 (71.43%) HLHS
TGA

Cardiac: Non-cardiac: False negative True negatives

3(50%) 3(50%), 1 (0.02%) 4144 (99.98%)
HLHS PPHN PA/VSD
Severe Ebstein anomaly Sepsis, pneumonia
Obstructed Cor-triatriatum

Results of pulse oximetry screening of newborns from Jan-Jun 2014 HKL 42

 Protocol for Newborn Pulse Oximeter Screening for Detection of Congenital Heart Disease HKL

Newborn participant eligible for screening:

•Place the mother’s sticker ID onto the log book At 0800 hr, a dedicated HO to copy all deliveries in labour room and LSCS
•Document baby’s: over the previous 24 hours onto the log book
-Gestation
-BW
-Gender A dedicated House Officer doing routine newborn checks in post-natal
- Age (hours of life) ward will place the Masimo Medical-7 pulse oximeter probe over any
oDate & time of birth
oDate & time of test
of the lower limb extremities of the baby & wait until a consistent
*test should be perform before discharge, (>6 hours reading and good pulse waveform signal is displayed.
of life).

Pulse Oxymetry reading >95%:

Pulse Oximetry reading < 95%

Warm up the baby’s feet

Repeat the measurement 1 hour after the
PASS first test
* Result will be documented
into a log book and baby’s
home base card
If Pulse Oximeter reading < 95% twice,
Medical Officer in charge of postnatal wards
should see the baby immediately, perform
High turn-over of HOs full physical examination and admit the
baby to SCN for close observation.
No Passover Identify cause of hypoxemia.
Screening discontinued ECHO if indicated
Inform case to specialist.
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Barriers & Challenges for Implementing Pulse Oximeter Screening

• No midwives / health care assistants with appropriate training in

postnatal wards

• Many babies are discharged before 24 hours of life

• No suitable pulse oximeter for babies in postnatal ward

• We have no cardiologist to echo

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We cannot cope, have no cardiologist to echo

Less false positive, less referrals

for echo

Train neonatal fellows to do

screening echo

After 2014, no babies with CCHD

delivered in HKL presenting in
collapsed state

For hospitals with no echo

facilities, referral should be
similar to existing referral system
for babies suspected of CHD

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AW Granelli. BMJ 2009;338:a3037
Basic Paediatric Echo Course – Hands-on
• 2009 – Hospital Kuala Lumpur
• 2012 – Precongress workshop –
Paediatric echo for non-cardiologist
14th APCP Kuching, Sarawak
• 2014 - Precongress workshop 15th
ASEAN Pediatric Federation Congress
Penang

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Conclusions
• Pulse oximetry is a safe, non-invasive, cost-effective, feasible post-natal
screening test for newborn infants with critical or cyanotic heart lesions
with good sensitivity and excellent specificity.

• It has better sensitivity than antenatal screening or clinical examination

• It also detects other diseases, such as PPHN, pneumonia, and sepsis early.

• Current evidence supports the introduction of routine screening for CCHD

in asymptomatic newborn before discharge from well-baby nursery

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Conclusions

• Pulse oximeter checks should be performed in all newborn babies in

the postnatal ward after 24 hours of life or as late as possible just
before discharge.

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Thank You for Your Attention

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