Neurodevelopmental and
Oral-feeding Outcomes for Infants
Sharon Sables-Baus, PhD, RN, MPA, PCNS-BC
Associate Professor, University of Colorado
College of Nursing/
School of Medicine, Department of Pediatrics
Table of Contents
Single-ventricle Physiology, Background (I)
Definitions (A)
Incidence (B)
Diagnosis (C)
HLHS: A Single-ventricle Defect (II)
Anatomy of Hypoplastic Left-heart Syndrome (A)
Physiology of Hypoplastic Left-heart Syndrome (B)
Surgical Repair of Hypoplastic Left-heart Syndrome (C)
Impact of Single-ventricle Defects (III)
Neurodevelopmental Issues (A)
Subsequent Feeding-related Outcomes (B)
Neurodevelopmental Research Foci (C)
Studying the Impact of Caregiving:
Sables-Baus et al, 2013 (IV)
Care Paradigm Utilized in Study (A)
Care Environment Utilized in Study (B)
Study Metrics/Methods/Objectives (C)
Study Results, Objective by Objective (D)
Implications for Future Research (E)
(I) Single-ventricle
Physiology,
Background
(A) Definition
(B) Incidence
(C) Diagnosis
 (I) Single-ventricle Physiology, Background
(I.A) Definitions
Examples of Single-ventricle
defects:
• Tricuspid atresia
• Double-outlet left ventricle
• Some heterotaxy defects
• Hypoplastic left-heart syndrome
 (I) Single-ventricle Physiology, Background
(I.B) Incidence
 (I) Single-ventricle Physiology, Background
(I.C) Diagnosis
Examples of Symptoms:
• Blue/purple tint to lips, skin, and nails
(cyanosis)
• Difficulty breathing and/or feeding
• Lethargy
(sleepiness/unresponsiveness)
A. Anatomy of Hypoplastic Left-heart Syndrome
B. Physiology of Hypoplastic Left-Heart Syndrome
C. Surgical Repair of Hypoplastic Left-heart
Syndrome
 (II) HLHS: A Single-ventricle Defect
(II.A1) Anatomy of
Hypoplastic Left-heart
Syndrome (HLHS)
1. HLHS—1/5000
babies, 1% of
congenital heart
disorders
2. Left ventricle
(pumping chamber)-small
3. Mitral and/or aortic
valves—narrow, blocked,
or not formed at all
4. Aorta—often small
(hypoplastic)
 (II) HLHS: A Single-ventricle Defect
(II.A2) Anatomy of
Hypoplastic Left-heart
Syndrome (HLHS)
 (II) HLHS: A Single-ventricle Defect
(II.B) Physiology of
HLHS
1. Deoxygenated blood flows into
right atrium, through tricuspid
valve, into right ventricle
2. From right ventricle, blood
pumped up to lungs, receives
oxygen
3. Oxygenated blood flows from
lungs into left atrium—unable to
pass into left ventricle (valve
blocked or narrowed)
www.ihm.org.uk
7. Gradually, sicker; ultimately, dies
6. When ductus arteriosus closes,
no oxygenated blood flowing in
baby’s body
5. While ductus arteriosus still
patent, blood passes from
pulmonary artery into aorta, then,
around body
4. Oxygenated blood passes through
hole between ventricles, mixes with
deoxygenated blood in right
ventricle, follows normal path to
lungs
 (II) HLHS: A Single-ventricle Defect
(II.C) Surgical
Repair of HLHS
(II.C1)
Norwood
(II.C2)
Glenn
(II.C3)
Fontan
 (II) HLHS: A Single-ventricle Defect
(II.C) Surgical Repair of HLHS
(II.C1a) Norwood
Procedure
Aim (ii) of the Norwood:
To provide blood flow to lungs
through Gore-Tex passage (shunt)
that links pulmonary artery and
aorta
(When: performed within a few days of birth)
www.ihm.org.uk
Aim (iii) of the Norwood:
To create permanent passage
(septectomy) between collecting
chambers (left and right atria),
ensuring that mix of oxygenated
and deoxygenated blood flows
throughout body
 (II) HLHS: A Single-ventricle Defect
(II.C) Surgical Repair of HLHS
(II.C1b) Norwood
Procedure
 (II) HLHS: A Single-ventricle Defect
(II.C) Surgical Repair of HLHS
(II.C2a) Glenn
Shunt
(Cavo-Pulmonary Shunt)
When: normally performed
between three and nine
months of age
www.ihm.org.uk
What (i): redirects flow of
deoxygenated blood to lungs
How: attaches superior vena
cava directly to pulmonary
artery
What (ii): Gore-Tex shunt
from Norwood taken away
 (II) HLHS: A Singleventricle Defect (II.C)
Surgical Repair of HLHS
(II.C2b)
Glenn Shunt
(Cavo-Pulmonary Shunt)
www.ihm.org.uk
 (II) HLHS: A Single-ventricle Defect
(II.C) Surgical Repair of HLHS
(II.C3a) Fontan
Procedure
When: performed around
three years of age
www.ihm.org.uk
What (i): separates
deoxygenated blood supply
from oxygenated blood
supply
What (ii): allows children
to grow, enjoy more
physical activity; does not
make heart function normal
 (II) HLHS: A Single-ventricle
Defect (II.C) Surgical Repair of
HLHS
(II.C3b) Fontan
Procedure
www.ihm.org.uk
III. Impact of
Single-ventricle
Defects
A. Neurodevelopmental Issues
B. Subsequent Feeding-related Outcomes
C. Neurodevelopmental Research Foci
 (III) Impact of Single-ventricle Defects
(III.A)
Neurodevelopmental
Issues
Peak
synaptogenesis of
medulla seen at 3436 weeks gestation
By 35-38 weeks,
nervous system
sufficiently mature
to carry out some
integrative
functions (e.g.,
nipple feeding
(clumsiness v.
coordination)
(drawing,
cutting,
handwriting)
 (III) Impact of Single-ventricle Defects
(III.B) Subsequent Neurobiological/Feeding-related Outcomes
(a) Single-ventricle in-utero physiology inhibits synaptogenesis/nervous system development,
leading to
(b) feeding challenges for the newborn child with surgery-necessitating single-ventricle physiology;
(c) corrective surgery results in
(d) a post-op recovery period during which oral-feeding challenges are heightened;
the (e) extended period of inadequate nutrition yields
(f) growth failure and
(g) adverse impact on cognitive and socio-emotional development, all of which yield
(h) parental anxiety and lack of confidence; parental anxiety and lack of confidence, in turn, produce
a tense atmosphere that heightens oral-feeding challenges, which cycles back to
(e) and then begins to loop.
(Einarson & Arthur, 2003; Lawoko & Soares, 2002)
 (III) Impact of Single-ventricle Defects
(III.C)
Neurodevelopmental
Research Foci
No studies to
date have
examined best
caregiving
practices postsurgery
IV. Studying the Impact of Caregiving
on Post-operative
Neurodevelopmental and Oral-feeding
Outcomes among Infants Born with
Problematic Single-ventricle
Physiology:
Sables-Baus et al., 2013
A. Care Paradigm Utilized in Study
B. Care Environment Utilized in Study
C. Study Metrics/Methods/Objectives
D. Study Results, Objective by Objective
E. Implications for Future Research
 (IV) Sables-Baus et al., 2013
(IV.A[1-3]) Care
Paradigm Utilized in
Study
(IV.A3) Early, consistent, high-quality
interactions to maximize
neurodevelopment, social-emotional
development, probability of school
readiness
(IV.A2) Thus, caregiving paradigm
similar to one used in NICU to improve
neurodevelopment and
neurodevelopmental outcomes
(IV.A3a) Early, consistent,
high-quality interactions
with clinical caregivers
(IV.A3b) Early, consistent,
high-quality parent-child
interactions
(evidence-based practice)
 (IV) Sables-Baus et al., 2013
(IV.A4) Care
Paradigm Utilized
in Study
(IV.4a) Profound
difference in
children’s
developmental
outcomes
(IV.4b) Profound
long-term savings
in human cost
(IV.4c) Profound
long-term savings
in social
expenditures
 (IV) Sables-Baus et al., 2013
(IV.B1) Care
Environment
Utilized in Study

(IV) Sables-Baus et al.,
2003
(IV.B2) Care
Environment
Utilized in Study
Helps families
find community
programs and
resources
Includes at homemonitoring
program
Offers screening for atrisk children with singleventricle physiology
Team includes MD,
PhD, PNP, CSW, RD
Provides
neurological/developmental
evaluation, diagnosis,
intervention
Helps educate community health-care
providers to increase understanding of
needs of children with CHD
Has effective tracking
mechanisms to increase
knowledge and refine
care for future patients
Administers
BSID every 3
months
Collects/shares data to establish international
guidelines for neurodevelopmental evaluation
and care of children with complex congenital
heart defects
 (IV) Sables-Baus et al., 2013
(IV.C1) Study
Metrics/
Methods/Objectives
Bayley Scales: An Example
Subtest
Composite
Score
Percentile
Classification
Cognitive
105
63
average
Motor
100
50
average
Age Equivalent
16 mos
Fine Motor
18 mos
Gross Motor
12 mos
Language
121
92
superior
Receptive
20 mos
Expressive
17 mos
Individually
administered exam
that picks up current
condition of
developmental delay;
does not predict
subsequent ability
levels, but triggers
anticipatory planning
of potential
intervention
strategies
• (IV) Sables-Baus et al., 2013
(IV.C2) Study
Metrics/Methods/
Objectives
Study
Methods
Chart review of 52 infants
with single-ventricle
physiology requiring surgical
repair, 8/2009-7/2011
Consideration of
medical/surgical variables:
gestational age, age at
surgery, weight at surgery,
cross clamp, DHCA/cerebral
perfusion time, length of stay,
days on ventilator
Consideration of
Bayley domains:
Cognitive, Speech,
and Motor
Consideration of oralfeeding variables:
consultation to OT/SLP,
consultation to ENT,
swallow studies, UGI,
feeding method

(IV) Sables-Baus et al., 2013
(IV.C3) Study
Metrics/Methods/
Objectives
a) To evaluate changes in
infants’ Bayley scores
from 6 to 12 months
b) To evaluate the relationships
between medical/surgical
variables and 12-month Bayley
scores
d) To evaluate the
relationships between
oral-feeding variables
and Bayley scores
c) To evaluate the relationships
between surgical variables and
oral-feeding variables

(IV) Sables-Baus et al., 2013
(IV.D) Study
Results, Objective
by Objective
First Objective
1. Bayley scores at 6-month follow-up: 20
2. Bayley scores at 12-month follow-up: 29
3. Bayley scores at both time points: 16
4. Data at both time points: paired t-tests used to compare
mean Bayley scores from 6 to 12 months
5. Marginally significant decrease in Cognitive and
Language scores over time; no change in Motor scores
Changes in Bayley Scores from 6 – 12 Months
Variable
Cognitive
Language
Motor
6-month
Mean (SD)
106.25
(16.38)
98.31
(13.10)
94.06
(21.67)
12-month Paired t-test
Mean (SD)
92.00
t=1.90
(28.81)
p=0.077
93.38
t=1.90
(11.53)
p=0.077
96.44
t=0.70
(18.32)
p=0.492
• N=16 infants tested at both 6 & 12 months
• Single LV=37.5% (N=6); Single RV=62.5% (N=10)
• Paired t-tests

(IV) Sables-Baus et al., 2013
(IV.D) Study Results,
Objective by
Objective
Second Objective
1. Variety of medical/surgical variables examined as
correlates of 12-month Bayley scores
2. No medical/surgical variables correlated with Cognitive
subscale
3. Evidence that greater total LOS, greater CICU LOS, and
greater number of days on ventilator related to lower
Language and Motor scores at 12 months
4. None of the other medical/surgical variables was related
to Language or Motor scores

(IV) Sables-Baus et al., 2013
(IV.D) Study
Results, Objective
by Objective
Second Objective,
continued
In addition, the following categorically measured surgical variables were examined to
determine relationships to Bayley scores using independent-sample t-tests:
1. Surgery category (arch & shunt; shunt & PA band; none [native PS])
2. Complications (yes or no)
3. Diagnosis category (single LV or single RV)
4. Pulse-Ox at discharge (yes or no)
None of these t-values was significant, indicating that none of the
categorically measured surgical variables related to any of the three
Bayley outcomes measured at 12 months.
Cognitive
Language
Motor
-0.08
-0.05
0.15
-0.003
-0.05
-0.13
-0.03
0.02
-0.10
0.02
-0.33+
-0.64***
-0.01
-0.38*
-0.55**
-0.12
-0.37*
-0.51**
0.13
-0.02
-0.01
0.07
-0.09
0.16
0.23
-0.12
-0.14
Gestational Age
Age at Surgery (in Days)
Weight at Surgery
Total LOS
CICU LOS
Days on Ventilator
Clamp Time
DHCA Time
Cerebral Perf Time
+p<.10, *p<.05, **p<.01, ***p<.001
Bivariate correlations of medical/surgical variables to
Bayley scores

(IV) Sables-Baus et al., 2013
Third Objective
(IV.D) Study
Results, Objective
by Objective
1. Surgical Variables:
•
•
•
•
•
•
Surgery category (Category 1 v. Categories 2 & 3 combined)
Complications (yes or no)
Diagnosis category (1 or 2)
Clamp time (yes or no)
DHCA time (yes or no)
Cerebral perfusion time (yes or no)
2. Oral-feeding Variables:
•
•
•
•
•
UMBS (yes or no)
ENT consult (yes or no)
Feeding method at discharge (breast and/or bottle v. other)
Enteral feeding (yes or no)
Bottle prior to surgery (yes or no)
UMBS
(% Yes)
Surgery Category
Category 1
Categories 2 or 3
Complications
Yes
No
Diagnosis Category
Category 1
Category 2
Clamp
Yes
No
DHCA
Yes
No
Cerebral Perf
Yes
No
ENT Consult
(% Yes)
Feeding@DC
(Breast/Bottle)
Enteral Feeding
(% Yes)
Bottle Prior
to Surgery
(% Yes)
32%
17.7%
34.6%
11.8%
23.1%
58.8%*
69.2%
70.6%
61.5%
64.7%
27.5%
0%
26.8%
0%
31.7%
70.0%*
70.7%
60.0%
60.98%
40.0%
9.1%
31.0%
9.1%
30.0%
63.6%
23.3%**
68.2%
70.0%
45.5%
66.7%
24.4%
17.7%
29.4%
5.9%*
32.4%
52.9%
64.7%
76.5%
58.8%
52.9%
30.0%
10.0%
29.0%
10.0%
29.0%
55.0%
64.5%
75.0%
67.7%
40.0%*
27.8%
18.8%
42.1%
9.4%**
26.3%
46.9%
57.9%
75.0%
52.6%
59.4%
Cross tabs were calculated to examine the relationships between the surgical
variables and oral-feeding variables. The table shows the percent responding
“yes” to the oral-feeding variables across the surgical-variable categories.
The * indicates a significant chi-square value (*p<.05, **p<.01).

(IV) Sables-Baus et al., 2013
(IV.D) Study
Results, Objective
by Objective
Oral-feeding Variables
Used:
• UMBS (yes or no)
• ENT consult (yes or no)
• Feeding method at discharge
(breast and/or bottle v. other)
• Enteral feeding (yes or no)
• Bottle prior to surgery (yes or no)
• Oral-feeding amount (total amount
divided by LOS)
Fourth Objective
Relationships of Oralfeeding Variables to 12month Bayley Scores:
• No oral-feeding variables related
to Cognitive scores
• ENT consult related to lower
Language (t=2.61, p=0.015) and
Motor (t=2.29, p=0.03) scores
• Breast and/or bottle feeding v.
other method related to higher
Motor scores (t=2.37, p=0.025)
• No other oral-feeding variables
related to Bayley scores
 (IV) Sables-Baus et al., 2013
(IV.E)
Implications for
Future Research
Need for Further Examination
of:
• Potential impact of timing of surgical repair
• Feeding experience as opposed to feeding
volume
• Specific caregiving practices
• Consistent parental presence—holding,
enfolding, olfactory inputs
• NIDCAP philosophy—physiological,
motor, and state systems
• Kangaroo care and pumping
*6th World Congress: Pediatric Cardiology & Cardiac
Surgery. Cape Town, South Africa, February 17 – 22,
2013: Neurobehavioral Outcomes of Infants seen in
Complex Congenital Heart Disease Clinic.
*Sables-Baus, S. (2013) Neurocognitive outcomes of
infants with single-ventricle physiology seen in
complex congenital heart disease clinic. Journal of the
South African Heart Association.