Tetralogy of Fallot Neurodevelopmental Outcomes

October 25, 2013

Tetralogy of Fallot

“Spelling It Out”

Gwen Alton RN, MN

Complex Pediatric Therapies Follow-Up Program

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TOF population

Complex Pediatric Therapies Follow-Up Program

– 1999-2008 registered the children with full repairs at < 6 weeks

– 2008- register children with shunts prior to full repair up to 6 months of age.

– 6 sites across western Canada

2000-2013n= 51

CPB(2000-2013)n=42(82%)

shunt(2008-2013)n=9 (18%)

Survival to 2y

n=37(88%)

Deaths to 2y

n=5(12%)

no chrom Abn

n=28*

Chrom Abnn=9

*

No Chrom Abn

n=2**

Chrom Abnn=3

Survival to 2y

n= 7(78%)

Deaths to 2y

n=2(22%)

No Chrom Abn

n=3

Chrom Abnn=4

No Chrom Abn

n=0

Chrom Abnn=2

Total survivors = 44 no chrom abn = 31 Chrom Abn =13 *ECMO

Acute Care Variables mean (sd); n(%)n= 51

Variables CPB n= 42 BT n=9Age (d) 39(36) 31(28)

Ventilator days 19(38) 9(8)

Hospital days 36(30) 26(16)

Sepsis 2(5%) 1(11%)

Convulsions 4(10%) 0(0%)

CPR anytime 2(5%) 1(11%)No significant difference between groups

Acute care variables mean(sd) n=51

Variables CPB n= 42 BT n=9

PO d1 inotrope score 17(12) 11(9)

PO d1 lactate (highest) 4(4) 2(1)

PO d2-5 inotrope score 16(8) 20(23)

PO d2-5 lactate (highest) 3(1) 3(4)

No significant difference between groups

Acute care variables mean(sd); n(%)

n=51Variables No Chr Abn n=33 Chr Abn n=18

Age (d) at OR 40(37) 34(29)

Ventilator days 11(12) 29(55)

Hospital days 30(22) 43(36)

Sepsis 2(6%) 1(6%)

Convulsions 1(3%) 3(17%)

CPR anytime 2(6%) 1(6%)

CPB* 30(91%) 12(67%)* p= .052 – trend to significance

Acute care variables mean(sd); n=51

Variables No Chr Abn n=33 Chr Abn n=18

CPB n=30 114(49) 139(59)

X-clamp n=29 56(26) 64(23)

PO d1 inotrope score 16(11) 16(13)

PO d1 lactate (highest) 4(4) 3(2)

PO d2-5 inotrope score 16(9) 19(16)

PO d2-5 lactate (highest) 2(2) 3(3)

No significance difference between groups

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Developmental ExaminationComplex Pediatric Therapies Follow-Up Program

• General health• Vision• Hearing• Language – expressive• Language

comprehension• Performance skills• Gross motor

• Fine Motor• Behavior• Social interaction• Intellect• Environment• Functional Behavior• Quality of Life• Nutrition

Multisite assessments

Multidisciplinary teams

9

Over view Outcomes n=44

Survivors n= 44

4 yearn=14

2 yearn= 21

Not yet seenn=7

LOSTn=2

10

Disabilities + General Health– n=35

• Motor: Cerebral Palsy n=2(6%) • Sensory: Vision loss n=1 (with CP)

Hearing loss n=1 (2.8%)(unilateral, not aided)

• G-tube at 2 year assessment n=9 (5 with Chrom Abn)• At 4 year assessment n=3 (2 with Chrom Abn)

• Hospitalizations # 3-9 in first 2 years (non-cardiac)

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Growth at 2 years: n (%) n=31

Anthropometrics No chrom n=23 Chrom abn n=8

Length < 3% 5(22%) 2(25%)

Weight < 3% 5(22%) 2(25%)

HC below -2SD 3(13%) 3(37%)

12

Outcomes at 2 and 4 years n=35

Outcomecombined 4y + 2 y

Mean(sd)

No chrom abn

n=25

Chrom abn

n=10

Cognitive 81.8(17.2) 69.3(20)

GAC (ABAS) 78(17) 68(17)

No significant difference between groups

No difference between CPB/BT group

GAC-General Adaptive Composite; ABAS - Adaptive Behavioral Assessment System

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TOF – outcomes n=35

Outcomecombined 4y + 2 y

Mean(sd)

No chrom abn/

Congenital

n=18

Chrom abn/

Congenital *

n=17

Cognitive 84(17) 72(19)

GAC(ABAS) 81(18) 69(16)

Quality of Life 85(18) 69(12)

* Skeletal dysplasia, VACTERL, neuroblastoma, dysmorphic

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Cognitive outcome– n=35

0

20

40

60

80

100

<70 70-84 85-114 115+

no chrom n=25

Chrom n=10

%

15

DEFINITION: Adaptive/Functional

• Level to which individuals meet standards of personal self-determination and social conscientiousness that is expected for their age, development and culture.

• Includes real life skills such as grooming, dressing, safety, cleaning, making friends, social skills

• Used in evaluation of children to determine strengths and weaknesses to help improve their success in school and life

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GAC – Functional outcome

0

20

40

60

80

100

<70 70-84 85-114 115

no chrom n=25

chrom n=10

%

17

COMPOSITE DOMAINS 10 SKILL AREAS

Motor

CONCEPTUAL Communication

Functional Pre/Academics

Self-Direction

SOCIAL Leisure

Social

PRACTICAL Community Use

Home Living

Health and Safety

Self-Care

General Adaptive Composite Score (GAC) includes all 10 domains

ABAS II Profile of Function

18

Skill area differences at 2 yr, m(SD)

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10

no chrom n=25

chrom n=10

NO CC n=13

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Early Developmental Intervention

0-3 years - is family centered Target groups:1. Established impairment – e.g. Down

Syndrome, Autism Spectrum Disorder2. Environmental risk – e.g. poverty3. Biological risk – e.g. early or ongoing

illnessAvailability: -parent requests - community -referral to tertiary level

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Early Developmental Intervention

Benefit – influences child, parent, family and reduces future concerns

Child Outcomes - social competence - emotional development - behavioral engagement - motivation

Parent Outcomes - resources, advocacy skills

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What can parents do to provide early assistance to their child?

– Read, sing, tell stories– Watch and listen to see how your child

communicates– Encourage your child to explore– Use words to help your child understand their

feelings– Give your child the chance to do things for

themselves – encourage to keep trying

Zero to Three – Early Learning

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Thank you

• All the families and children who have participated – Complex Pediatric Therapies Follow-Up Program

• Other sites across Western Canada• NICU, PICU, surgical staff – Stollery

Children’s Hospital• 4C staff & therapists – Stollery Children’s

Hospital• Glenrose Rehabilitation Hospital –

therapist and support staff• Co-chairs – Dr. Charlene Robertson, Dr. Ari Joffe, Dr. Reg Sauvé

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QUESTIONS